Ella's Corner

Catching My Breath (by Lindsay)

2012-03-10T14:57:00.000-06:00

We've had a very busy 48 hours. It's nice to finally sit down and have a chance to catch my breath.

The craziness started on Thursday morning...

It started like any other Thursday with aqua therapy at 8:30 am. I then took Ella to IKEA and The Container Store, trying to find some organization solutions for the giant piles of medical supplies that are accumulating around our house.

Ella and I then headed to Chicago for an appointment with her neurologist's nutritionist at Children's at 1:00pm. The appointment went very well. We discussed the new amino acid diet that Ella is on and the nutritionist was very encouraged to see that Ella is no longer sweating at all during the day (and much less at night). Ella's energy levels are also returning to what they were prior to her RSV and pneumonia. We're so happy to see that this new diet is allowing her body to digest the nutrients more effectively!

While we were at Children's, I got a phone call.

From Children's.

It was the sleep lab telling me there was a cancellation for a sleep study that night. After Ella's hospitalization last weekend, she was placed at the top of the sleep study cancellation list because we've been concerned that her BiPAP settings might not be optimal for her. But she needed to have a sleep study performed to try different settings.

So I made some quick phone calls to the pediatrician's office (getting referrals in place) and we headed back out to Naperville to pack up Ella's belongings, machines, etc. After saying a quick hello to Michael when we were home, we got back in the car and went back to Children's.

When we were about 10 minutes away from the hospital, I got a call from Michael saying that the referral for the sleep study was for the wrong hospital! And it was after hours, so we couldn't call the pediatrician's office to make the correction. The sleep lab said the only way we could have the study done was to pay for it ourselves upfront (yeah...not so much). Luckily, our pediatrician gave us her home phone number a while back (she rocks!) so I was able to call her and she made everything happen for us (she REALLY rocks!).

We got to the sleep lab and Ella got hooked up with all of the electrodes and she fell asleep around 10 pm. They tried the different BiPAP settings that we requested and she tolerated them well. We are still waiting to find out the results from Ella's pulmonologist.

What a GREAT way to travel inside the hospital...thank you for the wagon, Families of SMA!!

I showed this picture to Ella and asked her who it was and she said, "Monkey!" ha ha :)

Ready for bed.

We got up around 5 am and I had to replace Ella's ng-tube (I had to take it out the night before for the study). We gathered our belongings and packed up Ella's Bi-PAP machine and we headed back to Naperville.

Shortly after our arrival at home (and after Ella got a bath to clean off all of the goo from the electrodes), we got back in the car and headed to the New Lenox branch of Children's Memorial Hospital Outpatient center. We met with the surgeon who will be placing Ella's g-tube. She has to have an upper GI test done prior to the surgery, which will take place next Thursday (3.15.12). Her surgery will be on Tuesday, March 27th at Children's. We will plan to stay in the hospital at least one night, assuming everything goes well.

We then ran a couple of medical-related errands before returning home again in time for occupational therapy and then Ella's nap (during which time I was on the phone with doctors, nurses, receptionists, etc.).

When Michael got home from work, I was able to take a little break and go pick up my new glasses (mine broke in half the other day...go figure!).

When I got home, my mom had arrived with Ava and Henry...did I mention that my parents had them at their house since Wednesday night? Yeah, they TOTALLY rock.

Michael and I were then off to the appliance store to purchase a new range and dishwasher (we've been without a stove/oven for over a week and our dishwasher hasn't been drying very well for quite some time). We got back home in time to do Ella's cough treatments and get the kids to bed.

Finally, this morning we had an appointment at Mobility Works to get the final quote for the car modifications. Our next step will be to go to the Honda dealer to get the quote for the car. Once we purchase the car and bring it to Mobility Works, it will be another 4-6 weeks until it is completed. But we're getting close!

So that brings me here. Sitting on the couch, catching my breath. Henry and Ella are napping, my sister is making Shrinky Dinks with Ava in the kitchen, and Michael is helping my brother-in-law make an adaptation to Ella's small wheelchair that will help give her the extra trunk support that she needs :)

After I finish writing, I am going to attempt to start making room in our house for our ever growing mountain of supplies and equipment (by purging our house of anything and everything that isn't necessary...which is A LOT!).

Just a few of the things that we need to make room for...

And after I start making a dent in the home reorganization, I am looking forward to hopefully having some much needed down time in the near future :)

Our Friendships Are Now Hers...(by Michael)

2012-03-08T20:31:00.002-06:00

___________________________________

Years ago, many friendships were made.

And over the years...

They've witnessed happy times and hurtful times.

They've carried each of us through triumphs and tragedies.

They've comforted, consoled, and communicated.

They've endured the test of time and distance.

These friendships have once again been ignited to fuel the passion needed to show how deeply we care about the life of our Ella.

She is our Ella...she looks to those who befriended her parents years ago. She looks to these friendships to help her live her life to the fullest.

Click here for the Facebook Posting of this Event

A Sense of Peace...(by Michael)

2012-03-07T21:37:00.002-06:00

I read about a 10-month old boy who recently died because of SMA.

I received an e-mail from another SMA family telling me that they just had their boy in the hospital for 9 days because of SMA.

I read a Facebook post from a mother screaming out to cyberspace for a cure for this disease so her child could live...she was in such pain because of SMA.

I am part of conversations with others trying to figure out the best way to care for my child because of SMA.

It saddens me to know that a disease such as this exists.

It hurts me deeply to see my beautiful little girl struggle so.

I sit with her at night, as she lay in her bed, holding her hand. Her fingers are moving as she gains comfort from our connection both physically and emotionally. I can see her eyes behind her bipap mask; they're gentle and kind. She watches me watch her...I can see the reflection in her eyes of the screen and lights from the various machines...no longer does the moon cast its light upon her...for the moonlight has been overtaken by the glow of the machinery.

The sounds in the room are sounds one hears in the hospital. The humming of a small motor, the pulsing of a ventilator, the swishing of water that seems to keep the machines alive.

The machines' lights flicker and put on a show that provide messages of life...they are mesmerizing.

She lay still. Unable to move herself. Our dog lay faithfully at her feet. I know she'll need us to move her later in the night. I know that after looking at her as I enter the room I will quickly check the lights, the numbers, and the sounds for the normalcy I was taught to look for.

It's up to us to make sure she is at her best all of the time...day and night.

It saddens me to know that so many people, so many parents, have lost their beautiful child to SMA.

Early in the diagnosis, a father of a teenager with SMA Type 2 told me that one of the best things he did for his boy was to tell him that, "Everything will be all right" each night...that boy is still alive. I've taken that to heart with Ella. I tell her the same thing, if not with my words then with my eyes.

If nothing else I can do my best to give her a sense of peace...SMA cannot take that away.

Getting the Hang of It (by Lindsay)

2012-03-06T14:27:00.000-06:00

I took the kids outside today to enjoy this beautiful weather and to let Ella get some more practice in her power chair (and let Ava and Henry enjoy their new bikes!).

This was only the second time that Ella has had true freedom in her chair and I was surprised at how well she's starting to maneuver it!

I can't wait to get the new car so we can take her out in it all the time! We're getting close to officially starting the car process :)

I Am Not SMA (by Lindsay)

2012-03-05T21:19:00.004-06:00

Living with Spinal Muscular Atrophy is more than a full time job. There is so much involved physically, mentally, emotionally and spiritually. It truly is 24/7…I even dream about it (during the nights where we actually get more than a few hours of sleep in one stretch).

I don't think there are any words that I can think of to describe the challenge. Words that would describe the intense difficulty of living with this disease. And it only continues to get harder.

From the physical challenge of actually caring for Ella (including helping her with almost every single task she wants to do), to the emotional challenges of feeling the sadness of what she is unable to do physically and the fear of losing her. It's a lot to feel.

But lately, I've been letting SMA take over my life. And I'm not okay with that. Yes, it is a HUGE part of our life (and will be forever). But it's not our ENTIRE life. It can't be.

We still have our other children, Ava and Henry. We still have our marriage. We still have our families, friends and other interests outside of SMA.

But sometimes it's hard to draw the line between some of these things. Nearly impossible is more like it.

But if SMA is all we ever think about…if it's all we ever LIVE…then we're going to have a really hard time truly living.

It's going to take time, I know.

Sometimes it's hard to adjust because Ella's status keeps changing. Many times I feel that when I finally start accepting and feeling somewhat confident with where Ella is physically, she gets worse. And then I have to start over. And it continues to happen. And will continue to happen.

At some point I will hopefully be better at accepting these changes. Or at least be better at handling them emotionally.

But I CAN'T let it be who I AM.

I am not SMA. WE are not SMA. ELLA is not SMA.

Her Hold is Gentle Yet Firm...(by Michael)

2012-03-04T16:26:00.002-06:00

Ella waiting in the ER at Children's Memorial Hospital (March 2012)
with quite the sweaty head!

March 2, 2012. The temperature was dropping. Raindrops were changing gracefully into large fluffy snowflakes being blown in the winter's wind. The only person in our family who was sweating was Ella.

She has always sweated but only when sleeping. Prior to her pneumonia the sweating was of little concern since it was so mild. Post-pneumonia brought some new developments for Ella.

Since her discharge from Children's Memorial Hospital (CMH) on Feb. 20, after fighting the RSV and pneumonia, Ella has shown marked signs of apparent regression. We noticed her becoming heavier and seemingly more "limp" when we held her. The amount (and duration) of her sweating increased...from mild sweating while sleeping to a soaking sweat throughout the night and random head sweating during the day associated with very little physical exertion. The most disturbing development was her decrease in energies and abilities...she seemed to be going downhill rapidly.

Within the two weeks she had been home she gained one full pound. For Ella, this is too rapid of a weight gain in such a short period of time. Her muscles cannot afford the energy needed to sustain this rapid gain. She had the 1.5 Pediasure formula and needed the regular formula...in other words she was getting 1.5x the caloric intake.

The adjustment was quickly made by diluting her portions to accomodate the caloric intake she needed. While this slowed the weight gain, the problems of excessive sweating, lack of energy, and apparent regression persisted.

On Friday (March 2) just two weeks after being discharged from CMH we took Ella back to the ER.

Our concerns were simple.

The first being that we felt that her diet of Pediasure was not appropriate for her, being a child with SMA. Pediasure is higher in fat compared to other formulas. More importantly, though, is the type of fat it contains. There are long chain fats and short chain fats. Pediasure has long chain fats. These fats are very difficult for kids with SMA to break down and metabolize...short chain fats are much easier for their bodies to work with. There are other formulas that are lower in fat and are also short chain fats. The one that we were seeking for Ella is a formula called Pediatric Vivanex.

The second of our concerns lay with her Bipap settings (Bipap is her ventilating machine that she uses during naps and overnight sleep). The general idea behind the machine is that it assists with her inhaling and exhaling while she sleeps so she can conserve energy as well as fall into a deep (REM) sleep--while maintaining her O2 and CO2 levels. We would like to be sure that she is getting the optimal support specific for her.

After 5 hours in the ER, CMH admitted us to the PICU (Pediatric ICU) in order to monitor her while sleeping with her Bipap settings.

Ella waiting patiently in the ER (it's getting close to midnight)

They were looking for acceptable O2/CO2 levels. They explained to us that in order to change her settings she would need to be monitored through a sleep study. A sleep study will give us a specific picture of everything that is going on with her body as she sleeps. During a sleep study her Bipap levels can be tweaked to find her optimal support level while in a controlled environment.

Ella has a sleep study scheduled for early May but has been placed 'first' on the cancellation list through CMH.

The focus then turned to the sweating issue. We spoke to several doctors, nurses, "teams", and a nutritionist. We explained our concerns over and over. They were receptive and worked with us to solve this problem...or at least address it to maybe ascertain why the sweating was getting so out of control.

The bottom line was that we did not want to change more than one variable at a time. Lindsay and I felt strongly that her diet was playing an important role in all that we were seeing her go through in the past two weeks. We advocated for her that her diet needed to be adjusted to what we felt was optimal for her based on what we know about Ella, what we have researched, and the experiences of other SMA families. Digesting and metabolizing food can take a lot out of child with SMA...our call is to help her maximize her efficiency.

Ella had been visited by so many different people and she gave every one of them her signature smile. At one point she was hooked up to several wires while getting an EKG and chest x-ray to make sure her heart was working properly (they wanted to rule out heart problems causing the excessive sweating).

Ella hooked up for EKG while she sleeps early Saturday morning

In our 22 hour stay at CMH we ended up switching her to the Pediatric Vivanex formula. We are making the recommended supplementations (to her new diet) based on that fact that she is a child with SMA.

We are also posed and ready to work on optimizing her Bipap settings to give her the best breathing support possible as she sleeps.

Ella is a delight to be around. She is funny, easy-going, smart, and endearing. Her personality draws you in, takes a gentle yet firm hold of you, and places you at ease as the world around you is in chaos.

Ella getting ready to leave on Saturday morning

Ava & Ella missed each other...on Sunday morning they caught up with one another...

The girls...

Stop...by Michael

2012-02-27T06:39:00.001-06:00

Lindsay and I sat at the kitchen table late last Friday night. She told me that she wishes she could say, "When it rains it pours...", but she felt she couldn't say that with conviction.

The implication of this saying lies on two fronts. The first being that the rain represents something "bad" happening and the "pour(ing)" represents that it's just getting worse. The second implication rests with the first word of the saying: "When". This implies that there are periods of no rain...where things are calm.

Lindsay continued, "It seems as though it's always raining, always a downpour around here."

We looked at each other and shook our heads. Our gazes locked in a silent moment of questioning disbelief. What is happening to us? To our family? To our lives?

Earlier that night Ava became sick; she threw up all over the family room couch while I had Henry and Ella on my lap--on the same couch. Thankfully Lindsay's sister and brother-in-law were here to help. Between the four of us we isolated Ava, Ella, and Henry. We cleaned up the mess that was made in the family room. We cleaned off Ava and got everybody in their own space for the night.

That same night Lindsay told me about a conversation she had with Ella's OT. Her OT said that Ella's hands were becoming more seriously affected by the SMA. She's losing function in them. As Lindsay told me this my stomach was doing flips...I was feeling sick. I couldn't do or say anything except shake my head...I didn't know what or how to feel. Lindsay knew exactly what I was going through at that moment...she's felt it herself. It's a helpless feeling; surrounded by anger and despair, hatred and desperation.

Ella keeps regressing...we so desperately want it to STOP.

Since Ella's stay at the hospital, she is markedly weaker. She's harder to hold and she struggles more to keep herself sitting up. She has to put forth more effort to move herself in her manual wheelchair. Often times she asks to be pushed instead. It's impossible to watch her get weaker day in and day out. Every time we think we have figured out something for her...it changes on us.

We have set up our house with all of the equipment and are in the process of creating routines and schedules. Everything is different now. Timing is of the essence. It looks like a hospital exploded in our house. Add to this the normal chaos of having three kids, a dog and two cats...and you have a constant state of disarray. We know time will serve us well in that a sense of normalcy will prevail---we long for that...but until that happens...

The reality of what our family is going through and how we, as individuals, are coping with it continues to beat on us. We didn't really have time while in the hospital to think about all that was to be our lives when we got home...even when we did get home and had all of the equipment strewn about the place, we didn't have time to bring the reality close to us. Now that the house is basically set up and we are able to stop for a moment and look around, we are faced headfirst with the emotional downpour that surrounds us. Like Lindsay said, "...it never seems to stop."

To put into words what we are truly feeling at this time is next to impossible. Our feelings are at so many different levels, different layers that overlap. We are pulled in so many different directions, and for the most part we have no idea where they are leading us. The uncertainty of the future alongside the struggles of the present make for an experience that defies explanation. We are beyond numb.

"It's not real" we tell ourselves...but it is. And it's slowly chipping away at us.

We go on with our daily lives, doing what we have to. We do the best we can, just like everybody else, with what we have.

A Virtual Tour of Ella's New Equipment (by Lindsay)

2012-02-23T21:08:00.024-06:00

I thought I'd share some pictures and videos of Ella's new equipment in the house. It's been quite the lifestyle change for us adding these new treatments to her care and it will still take some time to get used to them (both on a physical and emotional level).

Here is a picture of our living room after her new equipment was delivered when we got home from the hospital on Monday (it doesn't include all of the supplies that we received, which we will continue to receive on a monthly basis).

Below is a picture and video of Ella using her nebulizer. She will most likely only use this when she is sick. It helps to break up secretions in her lungs, making it easier for her to cough them out. When she is sick, she will use this up to four times daily.

Here is a video of Ella using her "Vest" (a.k.a. "Shake"). I don't think I need to include much more explanation other than it helps to break up lung secretions. This is by far Ella's favorite treatment :) She does between 10 and 20 minutes for each treatment (in 5 minute sessions). When she is healthy, she will do this treatment twice every day. When she's sick, she'll do it four times a day. And yes, she "sings" pretty much every time :)

Below is a video of Ella using her Cough Assist machine with her suction machine. The Cough Assist machine blows air into her lungs, and then rapidly sucks it back out, simulating a cough (since she is too weak to effectively cough on her own). This treatment always follows a Vest treatment. The video shows one "cycle" of five breaths, followed by suction. Each treatment includes five cycles of at least five breaths. When healthy, the five cycles will be done twice a day, and when she's sick, four times a day.

The following picture is of Ella's NG-tube pump (nasogastric tube). Even though she is feeling much better, she still isn't eating. We're not entirely sure why she doesn't eat much (she didn't before she was sick, either). She is capable of eating, she just chooses not to. So whether it's due to weakness or something else, we don't know. But she needs nutrition. So she does an hour-long feed four times daily of Pediasure formula (sometimes we'll get a few bites of regular food in between tube feeds). Once Ella is completely recovered from the RSV and pneumonia (and her lungs can handle surgery), she will have a g-tube placed (in her abdomen). This will be a much more convenient way to do her feeds (and without having a tube sticking out of her nose!).

Next is Ella's pulse-ox machine. This one is quite simple. The probe is taped around one of her big toes while she sleeps and measures her beats per minute (BPM) as well as her oxygen saturation levels. An alarm sounds if her levels are out of the normal range for someone with SMA. So far, Ella's levels have been where they need to be.

Finally we have Ella's Trilogy, or Bi-PAP machine. This machine helps to make sure that Ella's lungs are filling to capacity while she sleeps. It also gives her weak muscles a break at night so she can get a better sleep and have more energy while awake. It doesn't actually breathe for her, it just assists her lungs. She uses this every night and at nap time, whether she is healthy or sick. She will always use it from this point on. It looks a little scary, but now that she's used to it, it's quite soothing for her and she falls asleep very easily with it on.

So as you can see, our little Squishy is quite the trooper. In a matter of 12 hours, she went from having absolutely NO medical intervention to having SEVEN machines that she will use on a daily basis for the rest of her life. We are so incredibly proud of our brave little girl :)

Time for Ella...by Michael

2012-02-21T20:29:00.002-06:00

Two hours she spent at the table.
She didn't want company.
She didn't want to interact.
She didn't want help.

She just wanted to play.

Nobody asking her questions.
Nobody hovering around her.
Nobody trying to make conversation with her.

Just to be alone. To enjoy life alone. To be alone with her own thoughts in her own world.

Ella wanted to be alone...

Six days and five nights at Children's Memorial Hospital was enough for Ella. While she enjoyed having so much attention given to her I think she really enjoyed coming home.

While at the hospital Ella had absolutely no control over her environment. She learned quickly that every time a person she didn't know walked into the room and was dressed a certain way (like a nurse or a doctor) that something most likely was going to happen to her...and it might hurt or be unpleasant. She learned quickly to say, "All done" or "Bye, bye" even before they could say "hello" to her. She also learned that they were exceptional people. They treated her with respect, kindness, and a gentle hand.

Even though she thought something was going to happen when a staff member entered the room; and even though she tried to get them to 'maybe' leave without having to do something to her; she knew that she did not have control over her environment or the authority to decide who was in it. At this realization she resorted to her charming self and everyone fell in love with her. It was a graceful and beneficial dance she did with the staff...they were able to do their all-important job with the skill and expertise they so naturally possess and Ella won over some more hearts!

When we returned home our house was quiet. Within a short amount of time the house itself was filled with equipment. Before she knew it there were an extra 5-6 adults in the house helping to get things in order.

Ella's fatigue and exhaustion kicked in. She grew impatient and cranky. She did the best she could as we had to set up (at least some) of the equipment and get her room rearranged for her slumber. Without the help of four men from church the night would have been a complete train wreck.

Ella was in bed and asleep before 9:00 pm...it was amazing on the part of those who helped us.

The next morning we did her therapy and sat her at the table to eat. Lindsay left for a few hours in the morning and I was home with Ella. I began straightening up the house and going through the myriad of bags we accumulated from our hospital stay and the fundraiser. I thought I might have 20-30 minutes tops to go through some of the things before I had to turn my attention to Ella...not so. I ended up with two hours!!

Two hours she spent at the table playing by herself...happy as could be.

She needed that alone time. She needed to be a regular 'ole kid just playing by herself without a care in the world.

Sometimes it's easy to want to be with Ella all the time...it's in our nature to want to help her. She was amazing to watch this morning as she played by herself, creating her own little world, not being bothered by the limitation SMA has placed on her.   There was a certain relaxation that came over me as I saw her out of the corner of my eye while I was busy organizing and cleaning. A calmness...an innocence...that only childhood can produce.

She needed her "Ella Time"...(a short video)...

Children's Memorial Hospital Pictures

2012-02-21T16:25:00.001-06:00

Since we were unable to post pictures with our blog entries while in the hospital, here are some photos we took during our stay...

Waiting in the emergency room.

Chest X-ray

First night in the hospital...finally fell asleep after midnight.

First cough assist treatment.

Resting after having her NG tube placed.

On our way to the PICU (pediatric intensive care unit).

Ella's room in the PICU.

First "Vest" treatment.

First time sleeping with the BiPAP.

Waking up after her first night with the BiPAP.

Heading back to the pulmonary floor.

Serious bed head.

Bath time!

Ahhhh, clean and starting to feel better :)

Whatever works for ya, babe :)

View from the pulmonary floor (not from our actual room).

Enjoying a chocolate covered strawberry.


Taking one last snooze before getting ready to go home.

Ready to go!

And for your enjoyment...Michael doing Cough Assist :)

Going Home (by Lindsay)

2012-02-20T10:11:00.007-06:00

We're just leaving the hospital.

Our feelings are surprisingly bittersweet.

I'm excited to go home. Living in a very cramped hospital room for the past 6 days has been rough. We haven't gotten much sleep. We miss our family, friends and regular daily life.

But I'm nervous. I'm nervous to go home. I'm nervous to be responsible for all of these new treatments that Ella will need on a daily basis. I'm nervous about how I will find the time to add these treatments to our already jam-packed days.

For now, this is what we'll be adding to our daily routine:

NG tube feedings 4 times per day (an hour for each session, not including set up/clean up)
Vest treatments 4 times per day (20-25 minutes for each session)
Cough assist and suction 4 times per day (15-20 minutes for each session)
Periodic pulse-ox checks throughout the day
BiPAP setup at bedtime every night (and possible adjustments/comfort throughout the night)

The only change once Ella is completely healthy (in 2 to 3 weeks) is that her Vest and cough assist treatments will only be once or twice each day, rather than four times.

We're also going to have to rearrange some things in our house. For one, we'll have to make room for all of her new equipment. Most of it will go on the first floor (except for the BiPAP, which will go in Ella's room).

We're going to have to figure out an organization system for all of her supplies. Not only will we have the equipment, but we will have all the disposable supplies that go with them. Tubing, tape, syringes, pads, masks and bags to name a few. And I'm talking a month's supply of these things, not just a handful. We'll have to figure out what will work best for our family and then start organizing (which, on the plus side, is something I LOVE to do). Dr. Prestridge said we should definitely invest in a label maker :)

Currently, Ava and Ella share a room. Ava is in a twin bed and Ella is in a crib. We're going to be switching Ella to a full sized bed so that we have easier access to her and her equipment and also so we can lie with her if we need to (the other night I slept with Ella IN her crib...I love our Squishy, but that's something I'd rather not do again if I can help it).

This means Ava and Henry will go back to sharing a room (for now anyway). We'll turn their twins into bunk beds, which hopefully will be fun and exciting for them :) And it will be better for Ella to have her own room anyway, since her machines will not always be quiet when she sleeps.

So while we're happy to be going home, we're a little apprehensive. We're leaving the safety of the hospital and entering a challenging world of daily medical intervention.

It's a little scary.

Ella's Day...by Michael

2012-02-20T08:04:00.006-06:00

The first hug that wrapped around each of us as we entered York High School on Feb. 19, 2012 brought with it tears. The last one as we left summoned the deepest feelings of love and gratitude that we have ever felt.

Ella's Day cannot be described in any other way than to say it was an incredible way for so many people to show their love and support for Ella and our family.

Over the past five and a half months, while the planning was taking place for this event, Lindsay and I met with the lead coordinator for a total of two times. She took the healm to orchestrate the event, showing the utmost in respect for our family and our emotional vulnerability.

After returning to CMH after Ella's Day, we sat in the hospital room with our Squishy, we were in awe of what we imagined must have taken place in order for this day to be the incrediblhy positive reality it was. We knew that so many people in our lives and a host of businesses came together to help. We knew that people who have no connection to us, apart from hearing or reading Ella's story, stepped forward in so many ways; pouring out their generosity. We knew that people spent hours upon hours coordinating, meeting, conversing, and making decisions on our behalf...on Ella's behalf. We knew that even though people have lives of their own, problems of their own, and struggles of their own they found time to focus their attention on Ella. We knew that Ella had found a place in the hearts of so many.

Despite our exhaustion from spending the three nights and four days at Children's Memorial Hospital prior to the event helping Ella battle her first case of pneumonia (which was brought on by RSV) we knew that being at Ella's Day was important to us for so many reasons.

Our lives changed upon learning that our youngest has SMA. Our daily life is (and will continue to be) strikingly different to say the least. Our perspective on life and what it holds for us has taken on a new meaning; one that includes the constant reminder that not only is life itself precious beyond our deepest awareness, it is also intertwined with everyone else's life in ways that can never be imagined.

Lindsay and I sat quietly with Ella as she fell asleep. The hum of her bipap machine softly telling us that it was doing its job was the only sound in the room. She had a hold of each of our hands as sleep began to overcome her. I often wonder what she thinks as she sits quietly with us. I know her mind is as beautiful as her spirit; and just as strong, too.

Lindsay and I will continue to hold fast to the promise we made to each other, the promise we made to Ella. We will continue to do our very best for her, for Ava and Henry, and for each other. That promise has been made easier to fulfill because of all the wonderful, caring people we are privileged to have in our life.

The day that was set aside for Ella has come to a close. The day that brought so many people together in so many ways has found its own slumber. The day that will forever bring memories and feelings of warmth, comfort, and healing to us has gracefully found its way into our hearts and will stay there forever.

Saturday Update (by Lindsay)

2012-02-18T14:27:00.004-06:00

Ella is continuing to improve. She's laughing and smiling and starting to get back to her old self (but she's still pretty weak).

Ella's right lung still has a little "squeak" to it, but it's much better from when she came in Wednesday night.

We met with Dr. Prestridge (Ella's pulmonolgist) this morning and it looks like Ella will be able to go home on Monday. Her new equipment will be delivered to our house that day, and as long as the home care specialists can come to set it all up for Ella on Monday we can go home then.

Tomorrow (Sunday) is the "Ella's Day" fundraiser in Elmhurst. As long as Ella continues to be doing well, our plan is for Michael and me to come to the event while my sister and brother-in-law stay with Ella in the hospital. Dr. Prestridge said this is completely fine and that the hospital has our cell phone numbers, should they need to contact us.

Just as a note, we have been writing these blog posts from our phones and we are unable to post pictures using the mobile version. Once we get home we plan to post the photos/videos from her stay (however, we have been posting some on Facebook already).

Thank you all again for your continued thoughts, prayers and encouragement. We can truly FEEL it and it helps tremendously.

Ella Update...Friday (by Lindsay)

2012-02-17T12:26:00.010-06:00

We've been at the hospital since Wednesday night. Ella has been having aggressive treatments to help her pneumonia and RSV symptoms, and she seems to be making some improvements.

They've taken her off IV fluids since she is meeting her nutrition goal with the ng-tube feeds (she still needs the IV for antibiotics).

She spent the night last night in the PICU (pediatric intensive care unit) for her bi-pap initiation, which went very well, considering it was her first time using the machine. She was able to return to the pulmonary floor today. Tonight she will use her own bi-pap machine that we will be taking home with us (last night she used the hospital's).

She's been using the "vest" treatment to help break up chest secretions (this can be used in place of the manual CPT/BD). Both of these treatments are followed by the cough assist machine. She's slowly but surely getting used to cough assist. It's an uncomfortable piece of equipment, to say the least.

We're not yet sure when Ella will be able to go home. She isn't going to recover any more than she has at this point for a couple more weeks, but we can continue her treatments at home to help her symptoms until she's recovered.

However, we have to be trained (and feel comfortable) on how to use all of the new pieces of equipment. Just a few minutes ago Michael and I used the cough assist and suction on our own!

We also have to have all of these pieces of equipment ready for Ella at our house when we bring her home. Some will be here (her bi-pap is already here for her to use tonight), but others are being delivered to our house. Once they are at our house, someone from the home assist company will have to set up the equipment for her (adjust sizes, settings, etc.). As of now, her Vest is being delivered tomorrow (Saturday) and her cough assist, suction, humidifier (for bi-pap), pulse-oximeter, and ng-tube pump will be delivered on Monday. They are trying to push for those things to be delivered tomorrow, but we were told it is very unlikely since it's so late on a Friday. So we'll see.

She will be keeping the ng-tube in place for feedings when we go home to make sure she's continuing to get the nutrition she needs. Once the RSV is gone in a few weeks she will have the g-tube surgically placed. The surgery is inpatient and will be done here at Children's.

Michael and I are tired and overwhelmed. We miss Ava and Henry (they can't visit because they are under 12 years old). But we wouldn't want to be anywhere else with Ella right now. We have no doubt that she is receiving excellent care, provided by professionals who are very familiar with SMA.

We're excited to go home, but nervous because of how different life will be. We will once again have to get used to our new normal.

And in time we will.

At Children's Memorial Hospital (by Lindsay)

2012-02-16T12:30:00.006-06:00

We're here. In the hospital. With Ella.

She has RSV (respiratory syncytial virus) which has led to pneumonia. It started as a simple cough/cold, but it is difficult for someone with SMA to cough up the secretions to fight off the infection, thus it can lead to a more serious illness.

So here we are. The pulmonary department of Children's Memorial Hospital in Chicago (however, we will soon be moving to the PICU).

We met with the entire pulmonary team this morning. Everyone we have spoken with is incredibly knowledgable of SMA (which is such a great feeling). Ella is in SUCH good hands here. The attending pulmonologist wants Ella to stay until her lungs are clear and she's stable enough for us to continue her care at home.

We were told to plan on staying at least through the weekend, if not until next week.

Wow.

So far Ella's treatments have included the following:

Oral antibiotics
IV antibiotics
IV fluids
ng-tube (nasogastric tube) for feeding
CPT/BD (chest physical therapy/bronchial drainage) every 4 hours
Cough assist machine (forces air into her lungs, then rapidly sucks it out, simulating a cough) every 4 hours
Suction to remove secretions from her lungs
Nebulizer treatments
Bi-pap machine (non-invasive ventilator) during the night. Soon she will be moving to the PICU for a few days to get this piece of equipment set up for her.

All of these treatments (minus the IVs) will continue when we get home (and most will continue on a daily basis even after she is healthy).

Once the infection clears completely (in a few weeks) we will probably have a g-tube surgically placed for feeding assistance.

So right now our goal is to get her lungs cleared using these aggressive interventions and have her become stable enough to go home.

I would say we're overwhelmed, but that doesn't quite do it justice.

Our lives are going to be completely different now. In the past 24 hours we went from Ella being independent of medical interventions to being dependent on a variety of pieces of equipment that she will use on a daily basis for the rest of her life.

I don't think we're going to know what to do when we get home.

Of course, Ella is being the trooper that she is, but there's no question that she's uncomfortable and doesn't enjoy being confined to her hospital bed. And saying that these interventions are uncomfortable for her would be quite the understatement. They will all take some getting used to.

Thank you for your continued prayers for a quick recovery for her.

What We Bring to Each Other...(by Michael)

2012-02-14T20:54:00.001-06:00

Ava has been intrigued by 'where we come from" or as she so eloquently puts it, "who made me (or mommy, or daddy, etc)?". Typical "children" questions that require tact and finesse on the part of the parent when answering.

Lindsay and I have approached this inquiry with a simple answer to the "who made so-and-so" question...our response has been, "God and 'this-and-that person'". For example...

"Who made Mommy?" asks Ava.
"God, Gramma, and Grandpa," replies Michael or Lindsay.

"Who made Daddy?" Ava continues.
"God, Yiayai, and Pappou," we tell her matter-of-factly.

And so it goes, Ava runs the course of our family history, interrogating us repetitively. Sometimes I can hear her under her breath reciting who made whom...of course, God is always the first name she speaks.

Recently, however, her questions have gone a bit deeper, Our four-and-a-half year-old is taking it to the next level. After confirming who has made whom she has now begun asking the "how" question.

"How did God, 'so-and-so', and 'so-and-so' make 'whomever'?" she ponders aloud.

My response..."With love".

She was satisfied. For a brief moment.

I knew it was coming but I was hoping maybe her silence was going to be a moment that would turn into some sort of distraction, taking her mind and thoughts elsewhere. Not the case. She persisted with the next logical question.

"How does the love make people?" she questioned with a tilt of her head and rise in her voice.

I looked at her; the beautiful eyes she has locking on my face; her expression seeking an answer; her silent patience surrounding us while I sorted through my mind's arsenal of words to use.

With a smile I said, "Love makes people through laughter, talking, hugs, and kisses. Love is what we bring to each other."

She smiled at me and went about her business.

Lindsay and I are fortunate people. We have each other. We have our respective families. We have our respective and shared friends.

We are fortunate in another way as well. We have our children. Three beautiful people who love us unconditionally. Three exquisite personalities that fill our house with energy. Three gentle souls that bring to us the innocence of childhood.

Our days are filled with Ava, Henry, & Ella. Our lives revolve around their care. Our hope and perseverance is fueled by their existence.

Our lives are filled with laughter, talking, hugs, and kisses...the "stuff" that made them is coming back for us.

Just a Month Ago...(by Michael)

2012-02-12T21:17:00.001-06:00

Where was I one month ago? Hmmm, mid-January. I suppose if I look back at my calendar I could probably figure it out.

Where were you?

This past Saturday (Feb. 11, 2012) our family went to the FSMA Illinois Chapter Meet & Greet. We went to meet other SMA families.

What we found there was so much more.

Dedication and love. We met with other families affected by SMA, we met with children who have SMA, we met with adults who have SMA, we met with people who have dedicated their careers to helping those with SMA. It was incredible.

Among all of these people there was one that stood out to me. I never actually met her yet I was touched (to say the least) by what she said during our lunch together as we all crammed into the adjoining conference rooms; she was in the room next to ours, separated by a large window and a door.

Her child died one month ago from SMA. Her tearful eyes shared her grief as she spoke; she was consoled lovingly by a man standing next to her, holding her shoulder as he shed tears.

One month ago her loss occurred and here she sits...with a roomful of people--probably many of whom she didn't know. She could have easily and blamelessly stayed at home this day. She did not. She came out in the bitter cold and met with people who still have their children; who still fight SMA. By being there she still fights SMA...despite her loss. She brought herself to a place filled with pictures of gorgeous children all fighting Spinal Muscular Atrophy in their own heroic ways. She placed herself in a roomful of people and let her vulnerability be known; trusting strangers with her thoughts, emotions, and heartache.

The room fell silent amidst her sharing. Waves of emotion passed through the door and the glass partition which separated the two rooms yet could not separate the people within the rooms. The tears of this mother and her companion spoke to every heart present there that day. Her bravery to be there, for whatever reasons she had, is a testimony to the love she has for her baby.

Just one month ago.

Rising Numbness...(by Michael)

2012-02-10T06:05:00.005-06:00

Ella takes Lindsay's hand just after her birth

The past week or so has found me feeling different somehow.

The look in Ella's eyes and the expressions she is beginning to make are grasping me more tightly than I expected they would. Having two other children, I know that a child's personality begins to manifest itself a bit more outwardly at about the age Ella is at right now. She is starting to interact in a way that speaks the message that she, as a person, is on her way to understanding the world around her. She is in the throws of sensing that she can make an impact by her actions, words, expressions, and emotions.

Ella's development has taken a slightly different route than Ava and Henry's did simply for the fact that she has SMA.

A month ago she may have looked at me with a look of guarded bewilderment due to her limitations...I might have felt discouraged not knowing how to deal with it. Now her eyes speak to me showing that she is beginning to understand; they say, "I want to do this but I can't." Falling into her eyes I give her a look that tells her that I am here for her...in any and every way she might need me.

A month ago she may have felt proud of something she did, as any child her age would. My reaction at that time would have an underlying feeling of "How long will you be able to do this thing?" Instead, I find myself holding her more closely to me with unspoken encouragement as she makes her way through the moments of her life.

There's a shift that has happened in my approach to being a parent of a child with SMA. A shift that I knew would come to me but did not know when. I am still in the process of shifting; mentally, spiritually, and physically. It will take time. I know.

The feeling that has stirred itself in me this past week has lain dormant for years. I haven't felt this way in a very, very long time. I never thought I would again.

The feeling does not fall in the spectrum of the what we normally consider each day in the face of turmoil...those of denial, anger, bargaining, depression, acceptance, and so on. The feeling that is playing its role in my life holds itself to a another set of rules; another way of expression.

I'm numb.

I'm numb yet I can sense the other feelings that surround my life--they are still there. They are, however, cloaked by a numbness that rises from deep within me. It rises and fills my body, mind, and soul. It allows me to face some of the terrible thoughts I have about the what is happening to Ella; without me losing control. It affords me the opportunity to speak to others about Ella, our family, and SMA. It generates a protective layer around me so that I can pray in thankfulness for everything I have that speaks of love. It is a necessary feeling; one that shuts down the other feelings, if only for a brief moment, to help keep my sanity in check.

I know that the numbness will subside. It will wax and wane. I know that the raw emotions that must surround me will do so in their due times...coming and going as they will.

The rising numbness must take its place in the traveling of the road I am on. It's a deep numbness that we all have within, waiting for its time to rise up and gather itself around us; to protect us, allow deeper reflection, and provide a hiatus from the tearing emotions that have been and will be.

Officially Vertical (by Lindsay)

2012-02-09T14:20:00.000-06:00

We got Ella's stander today!

Now we can start having her stand for longer stretches of time (working up to two hours per day).

This will help her in many ways, including increasing her bone density, helping her respiratory function, etc.

Next order of business...find a place in our house for another large piece of equipment!

Standing tall while enjoying a book.

Stand by me; Walk with me...(by Michael)

2012-02-06T20:34:00.000-06:00

She looks good, doesn't she?

Upright, feeling gravity but not succumbing to it, using muscles that have not long been used, standing.

She delighted in the prospect of standing and her face lit up when Lindsay placed her knee immobilizers in their respective places. We put her shoes on without her AFO's (Ankle-Foot-Orthotics) and let her have at it.

I snapped several pictures, each showing a slightly different angle and facial expression...this one captures her in a moment that she is more focused on her play than her standing...a feeling we want her to continue to have---just like you and I have. When we're engaged in something while standing, we really aren't thinking about standing...we're focused on our task. We want that for Ella.

Movement was the next logical "step" so we put on her AFO's and brought her to the dining room. While walking demands more from the body and gravity will delight in pulling one down during the task, we were right there with her.

She knows exactly what to do. It's amazing and quite encouraging. The motion of her legs is all her. Lindsay simply keeps her from falling...Lindsay takes care of gravity for her. Take that gravity!

The expression you'll find on Ella's face is one that we, as her parents, recognize as her expression of complete joy and pride, excitement and satisfaction. She wanted more. More vertical time, more walking time. We have to be careful not to wear her out since we know that's dangerous for her. We have to be careful how long we assist her as her dependence on us becomes more intense as gravity continues to grab for her...we don't want to injure her in the name of holding her up...it's a delicate balance between the experience her mind, body, and soul gains from this vertical time and the caution we must consider.

For now, however, enjoy a bit of walking time with Ella~~

Extra, Extra!!...Read All About It!!

2012-02-02T17:23:00.002-06:00

Ella has made the news and appears in
the February 2, 2012 online publication,

The Elmhurst Patch!

Link to the story:

Ella's Story: 'I'm Not Here to Save Her. I'm Here to Take Care of Her.'

Six-Month Review (by Lindsay)

2012-02-02T15:13:00.001-06:00

Today was Ella's six-month review with Early Intervention.

There were seven of us at the meeting, including myself, Ella's service coordinator, her physical therapist, her occupational therapist, her dietician, a developmental therapist and a social worker.

We all sat around the dining room table to discuss Ella's progress over the past six months. Well, progress probably isn't the right word.

It's no secret that Ella hasn't improved. Or even stayed the same. But that's the nature of SMA.

So we discussed what we've been doing and whether or not we want to continue or modify the interventions that have been in place.

After talking with Michael over the past few days, and then discussing further with the team today, we decided that we will be taking physical therapy out of Ella's weekly routine. We will be replacing it with a second day of aqua therapy each week.

Physical therapy is a challenge for Ella, to say the least. There's not a whole lot she can do. It is so difficult for her muscles to compete with gravity. But when she's in the water, she's almost weightless. She can kick, move her arms around, jump on the steps in the water, etc. (all while being supported, of course). And she loves it! So we all agreed that it would be much more beneficial for Ella to have two aqua therapy sessions each week instead of one aqua and one PT (however, the aqua sessions are done with Ella's physical therapist).

Another change we're implementing is to add a session of developmental therapy each week. In this therapy, we will be focusing on Ella's play. The therapist will give us ideas and strategies to make play time as appropriate as possible for Ella and also ways to incorporate Ava and Henry into her play.

Ella will continue to have occupational therapy every week, however, we are going to try to lengthen her time of massage and stretching (currently she tolerates about 15 minutes before becoming bored).

We also discussed some other modifications, pieces of equipment, etc. that she will be needing in the near future:

An adaptive chair for her to use at the table for meal times, therapy, etc.
A platform swing to install in the basement
A ball pit for sensory input
A Spio (compression garment to provide additional support and sensory input to stimulate her muscles)
Omega 3, coconut oil and other possible dietary supplements
A new activity table that we would build with holes cut out for "tubs" of toys, materials, etc. to try to make it easier for Ella to access items independently

While we know that Ella will not improve physically with therapy, our main goal is to help her become as independent in her world as possible.

Hush Little Baby...(by Michael)

2012-02-01T15:12:00.000-06:00

The mystery of sleep has fascinated the human race for centuries. We are now only tapping the surface of what goes on during our precious slumber. Spending a night with Ella during her sleep study proved to be a "wakening" experience.

We arrived at the sleep center at Central DuPage Hospital shortly before 7:00 pm. We were escorted to our room and immediately Ella took to her surroundings. The technician who was to conduct the sleep study provided us with everything we would need to help Ella be as comfortable as possible. Through every step of the process she explained what she was doing and why she was doing it in a calm, clear, reassuring manner.

I held Ella close to me on my lap on the large bed while her favorite video, "Sesame Street Sing Along" (dubbed "La La La" by Ella) was playing on the room tv. Our technician began the process of "hooking" her up.

Wires were placed in various places on her body; legs, torso, arms, face, head. Each time a wire was placed an explanation for its placement ensued. I was amazed at what science had learned thus far about sleep as well as how to monitor what is happening within the body during our most restful and vulnerable state.

Ella did not fight the wires. She did not cry. She did not complain. She sat contently in my lap, helped when she could, and talked when she felt she had something to add to our conversation. Her delightful and infectious personality shone through the circumstances that surrounded her.

Upon completion of "wiring" her I placed her gently into the provided crib. She looked up with her soft, gorgeous eyes and extended her petite hand to me~~not saying a word. I took her hand in my fingers. She held a grip on me that was impressive for her. She wasn't going to let the weakness of SMA stop her from holding fast to her daddy.

We said the "Lord's Prayer" as we always do before bed and I began to sing to her. Her favorite bedtime song right now is "Hush Little Baby"...I obliged...several times.

Her eyes fluttered shut after the third round of the lullaby yet she continued to pinch the skin between my thumb and index finger, something she does as a soothing mechanism for herself. I watched her slip into slumber; slowly, methodically.

There was one more sensor that needed to be connected to her abdomen. The CO2 monitor. This allows the technician to see the output of CO2 during sleep. If there is too little CO2 being expelled during sleep the body's cells get excited and wakefulness occurs. The machine wasn't working. A second machine was brought in. Same deal--not working. The technician reverted to her only option and had the CO2 being measured through a tube placed in her nose. The tube in her nose extends down toward her mouth to gather the expelled CO2 as she exhales. The tube gathers the CO2 and sends it to a monitor. Ella, however, sleeps with a pacifier. It blocked the tube from gathering all the CO2...therefore her readings were very low. The technician, however, needed to have a good amount of recorded sleep-time with the CO2 readings. Ours then became the task of removing her pacifier while she was in a deep sleep to get those required readings...timing was of the essence. All night long we worked to catch times when we could remove the pacifier for a long enough period. She was not at all thrilled with that.

Each time she woke, whether she did so on her own or because we were trying to extract the pacifier from her mouth, I would have to soothe her back to sleep. Of course, this meant that she would perform her soothing act of pinching the skin on my hand (between the thumb and index finger). Needless to say, since we had several wake-ups throughout the night, my skin in that area became sore...a small price to pay for our baby girl.

We traveled through the night hours, catching glimpses of sleep in between our wakefulness. Much of the time I found myself reviewing my life; replaying the major events of it, and how they intertwined leading me to this moment...the moment when I would peer into the crib that held our Squishy. And whether she was awake or fast asleep, I took in the beauty of her presence in our lives.

We will find out the results of the sleep study in a week or so. Whatever they may be is what they will be. We will take them for what they are and move forward accordingly.

Her grip was strong that night~~as is her personality, her bravery, and her character.

A Little Power Training (by Lindsay)

2012-01-31T13:43:00.010-06:00

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)

2012-01-29T16:28:00.001-06:00

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)

2012-01-27T16:31:00.001-06:00

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)

2012-01-27T06:41:00.000-06:00

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

2012-01-23T16:54:00.000-06:00

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)

2012-01-22T20:17:00.003-06:00

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)

2012-01-21T08:23:00.000-06:00

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)

2012-01-20T17:28:00.001-06:00

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 :)

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

2012-01-16T11:21:00.001-06:00

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)

2012-01-15T14:09:00.001-06:00

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)

2012-01-11T20:15:00.004-06:00

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)

2012-01-08T13:56:00.000-06:00

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.

2012-01-07T16:34:00.002-06:00

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)

2012-01-06T21:00:00.002-06:00

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)

2012-01-05T22:08:00.005-06:00

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.

Two Worlds (by Lindsay)

2012-01-02T20:55:00.000-06:00

Tonight as Michael and I were talking about SMA related things (a usual daily occurrence), we stopped and noticed how we're really living in two different worlds.

We have "our" world. The world we've been used to.

And then we have the "SMA" world.

Both worlds together now make up our life.

But they are still very separate.

"Our" world (the old world we were used to) included many things…

Our families.
Our friends.
Our church.
Our work.
Our day-to-day activities with the kids (preschool, play dates, etc.).
Other day-to-day activities such as running errands, doing laundry/housework, managing our finances, etc.

And then there's the new world. The "SMA" world. This world has a whole new set of things (not replacing our "old" things, but adding to them)…

Therapies.
Doctor Appointments.
Equipment.
Emotions. A whole new set of emotions that we didn't even know existed. That we didn't realize the human soul was capable of feeling.
Exhaustion

Like I said, so far these two worlds have been very separate. But I feel like they're going to have to start combining. At some point.

After all, BOTH of these worlds are a reality for us. BOTH of these worlds are part of our day-to-day activities.

But it's strange trying to incorporate our SMA life into life as we once knew it. Into relationships that used to be a certain way.

We've found that it is so easy for us to connect with other SMA families. We've been corresponding with several in particular over the past few months, all of whom have children who have been recently diagnosed, like Ella. They understand this new life. Completely.

It's sometimes hard to put into words what we're feeling. It's hard to convey the emotions involved with this type of diagnosis to someone who hasn't experienced it. Sometimes we don't want to. It becomes very easy to isolate ourselves from those who have been closest to us before SMA entered our life.

But these worlds are one and the same for us now. We can't pretend that one doesn't exist while we're wrapped up in the other. And that goes for both worlds.

So in time, we're going to have to figure out how these two worlds will interact with each other.

As uncomfortable as it is, we are going to have to figure out a way to mesh these two worlds together. We have to figure out how SMA fits into our "life as we once knew it".

Sigh...(by Michael)

2011-12-31T15:27:00.002-06:00

There could be a lot said for the past year, 2011. There could also be a lot not said. Looking back over the year in review I cannot help but sit back, take a deep breath, and sigh...

I sigh for lives lost and for precious lives gifted to us...

I sigh for dreams lost and for depth of soul gained...

I sigh for arguments waged and for forgiveness expressed...

I sigh for seemingly endless moments of fear and for comforts found in an embrace...

I sigh for rising anger within and for capturing peaceful glimpses of time...

I sigh for struggles endured and for victories celebrated...

I sigh for the loneliness that traps us and for the community that surrounds us...

I sigh for the chance to give and for the humbleness to receive...

I sigh for missing old friends and for the ones that once again emerged along with the new people filling our lives...

I sigh for my children's pain and for the healing hand of God...

I sigh for the exhaustion we've taken on and for the rest we find in one another...

I sigh for the people affected by SMA and for the gathering of loved ones at their sides...

I sigh for the world in all of its messiness and for the chance to experience its beauty...

I sigh for the challenges Ella has faced and for the strength in which she confronts them...

I sigh for the heartache my wife has endured and for the grace she showers over us...

I sigh for the darkness that covers our minds and for the light that shines in our spirit...

I sigh for every moment I missed and for each smiling gaze I took in...

I sigh for every tear shed in misery and for the laughter shared in joy...

I sigh for the year that has passed without pausing to give us rest and for the year that beckons us on with its promise of its hope...

To look back and define, quantify, or make a judgement on 2011 would be a exercise in futility. It was what it was; it brought what it brought; it allowed us to experience what we experienced.

To look ahead to 2012 and anticipate, hope, or otherwise seek something we have yet to experience is also an act of empty gathering. It will be what it will be; it will bring what it will bring; it will provide us with the experiences that it does.

There really is only the moment of now which is a gift, a present, to us. If we spend our time admiring the packaging it came in or speak only of the creation of the gift; or if we ruminate over what will become of the gift itself...we cannot possibly enjoy the gift for what it is...for it is right there in front of us and then it's gone. The gift, the present, is fleeting at best.

I know not what I'll sigh for in 2012...I do know, however, that it will be a sigh that draws in a deeper, more meaningful breath; designed to cleanse my mind, my body, and my spirit.

The Cough (by Lindsay & Michael)

2011-12-30T14:59:00.002-06:00

1 to 6.

That's how long Ella was up Thursday night; from 1am to 6am.

She has a cough. A simple cough...for you and me maybe, but not for a person suffering from SMA.

This poor little kid. She was coughing the whole time. Well, trying to cough.

Drawing in a breath strong enough to generate an exhale that will produce a cough capable of clearing her upper respiratory tubes is not something Ella can do. It's torture. And not just for the one who is suffering.

She was almost gasping for a deep breath. A breath that couldn't be there.

For close to 45 minutes Michael, in the wee hours of the morning, had Ella down in the family room performing chest PT (in an attempt to loosen up the phlegm in her chest) enough so her weak cough could have a better chance to expel properly. She lay on his lap, tummy down, head and upper body at a slight downward angle, and patiently let the chest PT (which is done on the chest, sides and back) do its work. She graciously allowed Michael to turn her on her sides and her back to complete the therapy.

When it was completed she wanted to watch TV. Michael put in her favorite Sesame Street video and sat her in her special chair in front of the screen. During the video she had a few good coughs and seemed to clear out some of the upper respiratory tubes. She laughed a bit and sang along with the songs.

Michael brought her into bed with us around 4 am. She tossed and turned, whimpering, until around 6 am, when Lindsay brought her back to her crib. Luckily she was able to sleep for a few hours.

We remembered what Dr. Lester (the pulmonary specialist) said... if she starts to develop a cough, we should put her on an antibiotic to try to prevent it from turning into something more serious.

So Michael took Ella to the pediatrician this morning (Dr. Murphy is out of town, so she saw Dr. Steinken). Dr. Steinken went ahead and gave her the prescription and told Michael the warning signs to look for that would indicate that she's getting worse (and that if she develops any of those symptoms, we need to bring her in immediately).

Dr. Steinken then asked Michael how WE (as her parents) were doing.

Michael replied, "Fine" (an automatic response these days). Then Dr. Steinken went on to say that with the diagnosis being so new, people who respond by saying fine are either in denial or hiding the truth. Hmmm...

He was right.

The diagnosis still IS so new. It sometimes feels like our entire lives have been about SMA, but in reality, it hasn't even been 5 months. Wow. How it has consumed our world.

That comment, however, was good to hear. So much of the time we feel like we should have moved on by now; accepted this awful disease and gotten on with our life.

But we're far from acceptance. Very far.

In an earlier post we spoke of the "phases" that go with life's events (DABDA) and we are finding out that often times the phases may overlap, producing a mix of emotions that sometimes may be at odds with each other. It's a mess. Life is messy.

Our wise associate pastor reminded us recently of the messiness of life; providing us yet again with something to hold on to. Here we stand again, at the crossroads of SMA and our life...seeking to do the best we can to keep our Squishy healthy.

This whole "SMA ride" has been just that... a very strenuous ride of ups and downs. And then more ups and downs.

Today we are down. Very down.

But hopefully Ella's cough will be minor for her and not develop into anything more serious.

And then hopefully, at some point, we'll go back up.

Excited Sadness...(by Michael & Lindsay)

2011-12-27T22:21:00.004-06:00

Three-hundred eleven pounds.

15x the weight of Ella.

Designed in Sweden.

Astronomical price tag.

Delivered to our house and received with mixed emotions.

The Permobil K450 MX is here.

Having this piece of machinery in our house for our 18-month old daughter strikes many chords with us.

We are excited to have it because it means that Ella now has a long-term solution to her lack of mobility but it carries with it the reminder of what SMA is doing to her body.

We are grateful that modern technology has equipped Ella with a state-of-the-art wheelchair that will allow her to experience the world in a way she hasn't yet and might not have without it. It brings with it the awesome task of preparing her, Ava & Henry, and ourselves to live with this monster of a machine in our living spaces.

We are in awe at the capabilities the Permobil boasts; it's versatility, power, and parent-friendly controls. It calls us to the reality that our house will not for long be able to accommodate Ella and her needs.

The excitement in her face when we were making the final adjustments to the chair right before she was to sit in it lit up her cheeks. She eyed it from as many angles as she could manage and sat willingly in the tiny chair designed just for her.

She took command of the controls almost immediately. Seeking to explore the new found experience that lay before her. The buttons delighted her. It's capabilities enthralled her. The sheer magnitude of it enveloped her as she gathered ever increasing confidence with every moment she was in control.

The training that will be required captured our attention as we watched how she attempted to navigate the tiny room she was in. This will be a task among tasks to teach her how to maneuver the 311 lb. chair with her delicate 1.5 inch fingers. It will take weeks, if not months, before she will be truly independent in this chair. Not to mention the fact that we have house & car modifications that will require time before they are completed.

Looking at the Permobil K450 MX boggles the mind. It tells of a society that has advanced itself. It boasts of a society that has dedicated itself to those (within the society) that need help.

It also brings to life the reality of why it exists.

It excites and saddens us that Ella has this in her life.

Oh...by the way, we've decided this new chair needs a name. Any suggestions? If so leave a comment...

The Regression Continues (by Lindsay)

2011-12-26T12:50:00.001-06:00

Earlier in the fall we learned that Ella's continued regression was not usually "typical" for a child with Type 2 SMA, which led to the new classification of "severe Type 2." However, we were told that at some point (hopefully in the near future) her regression would plateau for the time being.

About a month or so ago, we started thinking that the regression had indeed slowed, if not stopped. We were very excited that we could finally start focusing on the skills she had, not worrying about what would be gone tomorrow. Or the day after that.

But then a couple weeks ago, Ella all of a sudden lost the ability to lift her arms at her shoulders. She used to be able to lift them to almost 90 degrees. She can, however, still bend them up at her elbows.

I told her PT about our concern last Tuesday, and she immediately saw what we were talking about. Ella couldn't reach for anything. She also noticed that Ella's neck muscles seemed weaker because her head was occasionally falling and she couldn't get it back up.

Lynda (her PT) had me video tape this new regression and email it to Dr. Silver (neurologist) that morning. A couple hours later, his nurse called me and asked if we could bring Ella in to see him that afternoon. We did.

Dr. Silver was concerned that something else could be going on that would cause this sudden additional regression. Maybe she was not feeling well. We thought maybe even her broken arm could have been part of the problem, but that was ruled out because she was able to lift her arms even when she had the heavy cast on.

There was nothing else wrong with Ella. Dr. Silver put it quite plainly... it's just the SMA. She's just continuing to regress. His actual words were, "Her anterior horn cells are deteriorating more rapidly than we would like to see."

Well fabulous. Just what you always like to hear about your baby girl.

So now we continue to go on. Hoping and praying that her regression will plateau soon. It is so very difficult to see her struggle so much on a daily basis, but what makes it even harder is to see her get worse in such a short period of time.

Please continue to pray for our Squishy.

How Does This Happen Anyway? (by Michael)

2011-12-23T14:43:00.007-06:00

Most people I have spoken with since Ella was diagnosed with SMA have told me that they had never heard of this disease.

Neither had we.

Yet it remains one the most devastating genetic diseases out there.

How does Spinal Muscular Atrophy happen anyway?

The answer lies in the genes. A single protein.

Here, it looks a little something like this...

The SMN1(Survival of Motor Neuron) gene is what people with Spinal Muscular Atrophy are missing. This gene produces the protein that keeps our motor neurons alive.

The SMN1 (Survival of Motor Neuron) gene is located on chromosome #5.

Mother nature produces two of these genes on chromosome #5:

*an original (SMN1): This is missing in people with SMA

*and copies (SMN2): People with SMA can have up to 6 copies

The two models below show how SMN1 and SMN2 genes make their proteins and how Spinal Muscular Atrophy occurs:

Normal SMN1 protein production
(model below)
proceeds as follows:

(a) The DNA is "spliced" by the RNA
(b) The RNA then translates the spliced parts making a protein using 8 full portions (called exons)
(c) The mRNA transcribes the entire protein (using all 8 exons)
(d) A normal (functional) SMN1 protein is produced.

__________________________________

Since people affected by SMA do not have the [original] SMN1 gene they are reliant upon the [copy] SMN2 gene to produce the needed protein.

Spinal Muscular Atrophy occurs (on the SMN2 gene) because:

(see model below)

(a) The DNA is "spliced" by the RNA
(b) The RNA then translates the spliced parts BUT the #7 portion (exon) is deleted or mutated, making an insufficient protein.
(c) The mRNA transcribes the protein without the #7 portion (exon) or with a mutated form of it.
(d) An "Unfunctional" SMN2 protein is produced: SMA Results

In short: People with SMA do not have the original SMN1 gene (therefore they do not produce the protein needed for their motor neurons to survive).

They do, however, have back-up copies of the original gene called SMN2 (some people produce more than others: Our Ella has three copies.)

The problem with the back-up copy however is that it is missing or has a mutated key component (exon #7).

If you look at the RNA (b) part of the picture above you will see that while the SMN2 gene is "splicing" it skips or mutates the #7 portion (exon) of the gene. This skipping/mutation produces an "unfunctional" SMN2 protein...Spinal Muscular Atrophy is the result.

The SMN2 protein is not sufficient to keep the motor neurons alive for very long and they slowly die off; hence the progressive, degenerative aspect of SMA.

Researchers' Approach:

Since researchers know that the problem with SMN2 is the deletion (or mutation) of the #7 exon, they are hoping to intervene (with a new experimental drug call SMNRx) at the RNA (b) splicing point--telling the gene to not skip or mutate the #7 exon thereby producing a more functional protein.

A pharmaceutical company, known as ISIS, is set to begin phase 1 clinical trials using SMNRx.

Clinical trials occur in 4 phases:

Phase 1: Researchers test an experimental drug or treatment in a small group of people (20-80) for the first time to evaluate its safety, determine a safe dosage range, and identify side effects.

Phase 2: The experimental study drug or treatment is given to a larger group of people (100-300) to see if it is effective and to further evaluate its safety.

Phase 3: The experimental study drug or treatment is given to large groups of people (1,000-3,000) to confirm its effectiveness, monitor side effects, compare it to commonly used treatments, and collect information that will allow the experimental drug or treatment to be used safely.

Phase 4: Post marketing studies delineate additional information including the drug's risks, benefits, and optimal use.

Courtesy of: http://clinicaltrials.gov/ct2/info/understand

Calendars (by Lindsay)

2011-12-22T16:54:00.000-06:00

Yesterday I got our new 2012 calendar.

In addition to our online calendar, we have a wall calendar that I write pretty much everything we do on. It hangs on the side of our refrigerator, so I can have quick, easy access to it.

The first thing I do when I make the switch from one year's calendar to the next is go through and write in all the obvious dates (birthdays, anniversaries, etc.). I have the two calendars side-by-side, flipping through each month together. Then I check our online calendar and go through and write any appointments or events that we already have scheduled for the new year.

While going through the 2011 calendar, I found myself noticing the strange progression of our year. Early in the year, things were fairly uneventful. But then when the summer months hit, our weeks started filling with more and more doctor appointments.

And then I came to August 12, 2011. That dreadful day that I will never forget. The day our lives were turned upside down.

It was hard to take my eyes away from that small white square on the 2011 calendar.

It was weird looking at the day before (the 11th, our anniversary). We didn't know. Yet. We had a good suspicion, but we didn't know for sure.

And then the weeks really started filling in. More doctor appointments. PT, OT and aqua therapy every week. Dietician appointments every month.

It was such an odd feeling looking back to those early months of 2011, when our calendar was so empty. What did we do? How did we spend all of that time?

How did we feel? I honestly can't remember what it felt like to not have SMA in our life. I don't know if that's good or bad.

I do remember writing out all of the 2011 dates in our calendar a year ago. I sat in the kitchen. Today I'm in the dining room. But a year ago, I had no idea what was really in store for us.

Who would have ever thought we'd be where we are now?? Our baby was a perfectly healthy, normally developing little girl.

I wonder what 2012 will be like for us. For Ella. I wonder what our life will be like a year from now, when I'm filling out the calendar for 2013.

I'm hopeful that good things will happen for our Squishy. For all of us.

Just Around the Corner...(by Michael)

2011-12-20T19:11:00.000-06:00

Christmas is just around the corner.

This year is different. It has called Lindsay and I to look at our lives and the lives of our children in such a different way.

The “holiday spirit” of yesteryear brought with it the gathering of family & friends, the gifts, the lights, the food, the decorations. All designed to enhance the feelings associated with the season.

It’s different now. We do not know how many Christmas’s we’ll have with Ella. That’s a stark reality that brings our world to a halt whenever we think about it.

Everything outwardly is still the same; the world goes on with the preparations, the merriment, the anticipation. We have found that the change is inside of us. We see the season with new eyes and a virgin heart.

Yes, we got a tree. Amidst the decorations and lights it seems empty somehow. It's there yet it stands alone. Kind of sad in a way. When we gaze upon our tree the feelings elicited draw us deep into the moment we find ourselves; the life we now have. How different this life is and will be.

Yes, we talked about what to buy for whom. It seems we cannot focus long enough to find the right gifts. Our minds quickly turn to the needs of our children; their ever-increasing needs. Our thoughts are captured by the reality of the financial burdens that have crept their way into our nice little budget we had worked so hard to develop and make work. We search for meaningful gifts within our means; seeking them to truly be from our hearts to the hearts of our loved ones. What might those be?

Yes, we made plans with our family. They know not how much we need to rest in their love. Spending time with each person this holiday season will keep us connected. We feel cared for by them in a way that no other group of people can care for us. They say one cannot choose their family...I don't believe that...I think we chose each other long ago.

Yes, we have attended church. The depth of compassion, support, acceptance, and love we have found at St. Timothy's has held us strong. We have attended the church classes centered around the Nativity. In this we have found something. Our inward change finds its roots here...

We feel the hand of God reaching out for us...showing us there’s now so much more to Christmas than what we have been celebrating in years past. We will not give up the traditions and celebrations of the past...we will simply enhance them with what we are feeling in our souls to a greater degree. There is something deeper...something that we know will reside within us, even after the holiday has passed and until it reappears again the following year, if we allow it .

Nothing else really matters to us more than the life of our children, especially that of our Ella; whose life is threatened daily by SMA. Watching her lose her ability to sustain herself physically shreds one’s heart into pieces, steals those pieces, and scatters them flippantly back onto the soul…we look to God and each other to gather up those pieces and restore them to their proper place. We embrace those who seek to comfort us. We walk through this life with a numbness that defies explanation...a deep hole if you will, that surrounds each of us…black, darkened, and void of escape on our own.

There’s only one way out and we are finding Christmas speaks to us of that promise.

Lights and decoration will come and go. Gifts will find their rightful place within our lives. Food and preparations will be distant, pleasurable memories, among all the others.

The long-lasting eternal meaning of what we now experience through Christmas is based on hope, perseverance, and gratitude. As peacefully and perfectly as God entered this world for us shall He enter our hearts and lives. We’ve found that putting aside all earthly cares has opened up ourselves to receive.

Christmas is right around the corner, yet it brings us to a path filled with a deeper understanding of life that we now must continue to travel...for the sake of our children, the sake of ourselves, the sake of Ella Sabine.

I Care (by Lindsay)

2011-12-19T16:54:00.000-06:00

Today I care.

I've found it's been very difficult to care about much these days.

It's hard enough to just make it through each day.

My to-do list has been getting longer and longer, and I've just wanted to crawl in a hole until those tasks just vanished.

But guess what? That's not how it works. Go figure.

But something came over me today and I was productive.

I did laundry. This is typical for any given Monday, but I still did it.

I cleaned. I mean REALLY cleaned (the first floor, anyway). This is not something I've done in a while. It's been one of those things that I didn't care about. At all. But every time I'd walk by a some junk piled up high, or dirt and dust on the floor, it would make me angry. But I never did anything about it, because I didn't care enough. But today I did.

I called two places to start the process for getting a lift installed in our car for the new wheelchair. This was a very simple task, yet I hadn't done it yet because I didn't care. I didn't want to start the process. I know it will require more work, and that's the last thing I've needed. But it's been started. And now I just have to wait for them to call me back.

I also called the two companies to get estimates for a ramp for our house. Once again, not something I've wanted to do, but I did it today.

I called Children's Memorial Hospital to have Ella registered. She's going there to see a pediatric neurologist (Dr. Nancy Kuntz) on January 5th. We've heard of this incredible doctor through Ella's PT and also through FSMA. We've been told that she has a phenomenal SMA team and that she is very proactive (whatever that means for a disease with no treatment or cure). But we were curious. So we asked Dr. Murphy if we could go see this doctor, and she made it happen.

In the past, completing tasks was just a part of daily life. I never thought much about it. But now, things are different. Motivating myself just to get out of bed in the morning takes a whole new kind of energy and strength. I use up everything I have just to take care of Ava, Henry and Ella's immediate needs. Tackling to-do lists is so incredibly difficult.

But today, I did it. And I feel good.

18 Going On 4 (by Lindsay)

2011-12-16T16:31:00.000-06:00

This week I've been able to reconnect with a few close friends. I've definitely been "out of the loop" lately, so it was very nice to be in such good company again.

Many of my friends have kids the same age as Ava, Henry and Ella (or very close). It's always been great for us moms to catch up, while the kids entertain each other.

Today, my good friend and I listened while our children (Ava, Henry, Sophie & Luke) played hard. They went down the basement and played on the "roller coaster". Then they all ran upstairs to the second floor to jump on the beds (and somewhat destroy the nicely arranged rooms... sorry, Mary!). Then it was back to the basement, and back upstairs again. This happened for a good 3 hours (with a little lunch break in between).

But there were two other children at the house on this sunny December day.

Ella and Maggie.

Ella and Maggie are good buddies. Ella is 18 months old. Maggie is 4 months old.

Ella is almost 5 times Maggie's age.

Yet they both see the world from a very similar view.

Ella wasn't able to join in the fun with her sister, brother, and their friends. She can't run up and down the stairs. She can't jump on and off the bed. But she was very happy playing with "baby Maggie", as she loves her very much. And Maggie stays in one place on the floor. Just like Ella.

But what happens when Maggie starts crawling? And then walking and running and jumping?

Ella will still be lying on the floor.

Yes, she'll have her wheelchair. But it won't be the same. The kids will still run up and down the stairs and go places where her chair will not be able to go.

I'm sure we'll get creative and figure out ways and places for the kids to play so that Ella can be part of the activities. We'll have to.

But in the meantime, I'm so glad that Ella has her Maggie :)

Don't Be Fooled...(by Michael)

2011-12-15T20:50:00.001-06:00

Don't let the scene above fool you.

Yes, the three Casten kids are engaged in their own separate activity.

Yes, the room is quiet.

Yes, this only lasted for about 10 minutes.

Raising 3 kids under the age of 5 is something we knew would be challenging. We knew they would fight, we knew they would bother one another, we knew they would make bad choices right alongside their good ones.

Don't let the scene above fool you. They are just about driving us crazy.

While SMA first and foremost affects Ella, it digs its claws in the whole family.

Ava and Henry are unable to express, in a way that is productive, the feelings they encounter internally and externally in regards to SMA. But they do have those feelings. It's very hard for them. Our task is to identify when they are overwhelmed by life...and help them through it.

Ella is a smart girl. She is beginning to understand that her disability forces her into a different way of dealing with the world. She has no model. The older she gets, the more she'll understand...a blessing and a curse.

For right now, though, we are doing our best to sort through what life has handed us. We feel numb. We feel defeated at times. We feel that the enormity of caring for our children will consume us.

The scene above is one that we must hang on to...it reminds us that there are three individuals; separate and distinct from one another; whose life, health, and well-being depends upon those who love them.

Don't let the scene above fool you...it really is a calling for us.

DABDA...(by Michael)

2011-12-13T16:30:00.003-06:00

I hold a BA in Psychology and an MA in Education.

I am fascinated by human behavior. I have learned much through my life experiences, my education, and from working directly with children & families for nearly 20 years now.

I remember learning about the 5 emotional phases that people go through when dealing with difficult times. I recall them with the acronym "DABDA":

*Denial

*Anger

*Bargaining

*Depression

*Acceptance

People go through these emotional phases when dealing with all kinds of events, although they may not necessarily be experienced in the order written; and often, any one of them can repeat themselves within the given event.

Within the past three days I have been angry. It's not something that I easily admit...at least not publicly like this, but it is true.

It’s an anger that resides deep within my core. An anger that works its way into the fabric of my life. An anger that I am fully aware of and at the same time I fight to keep down. An anger that is persistent, powerful, and unforgiving; feeding on itself; delighting in my pain. This anger, which is part of the emotional spectrum, is met with all my other feelings; vying for a position of highest emotion. It is strong. It is there. It is making a break for me. It demands my attention.

I know it is healthy for me to experience this emotion; as negative as it may be. I must honor the spectrum of emotions...I must allow myself to feel all of them. I get to know my emotions so I can deal with them appropriately. This emotional war has been thrust upon me.

I sat in church this past Sunday. I listened to the pastor as Lindsay and I kept our eyes on our three little children. I have become quite good at giving the pastor my ear while being summoned by the wants and needs of a 4 year old, a 3 year old, and a one-and-a-half year old.

The sermon held its focus on the idea of hope. It was delivered alongside the ideas of optimism and pessimism. He related the three to one another to further illustrate his point.

I walked away with a better understanding of how optimism and pessimism wax and wane—how at any given moment they may come and go; depending on external factors that we live with everyday. Hope, on the other hand, does not ride the same waves optimism and pessimism ride.

When he spoke of hope I found myself listening more intently. My mind took in the idea that hope rides the winds of life in a upward, smooth, and ever increasing motion. It glides along our experiences, providing the impetus to persevere.   It, unlike optimism and pessimism, is not dependent on external factors. Hope is a result of our soul’s need to let God in. Once we let Him into our hearts, truly let Him in…hope springs eternal.

Anger heard the same words I did. We battled for days. Anger took shots on me at every turn. Battles ensued.

I have fought with anger before in my day. I'm scarred, to say the least.

I've also learned about my enemy: my anger...I know its weak spots. I have armed myself with the one weapon I know will defeat it...

Love.

For with the love of my wife, the love of my children, and the love of all those who surround me anger stands not a chance. It will come again. It will find a place and a weakness within me...it will strike. I will fight once more.

I have love...and from that love I have hope. And within that hope I place myself.

She's Free! (by Lindsay)

2011-12-12T16:55:00.000-06:00

I always wondered how they got a cast off...

Today I got to see how it happens. Ella got her cast off and had new x-rays of her arm, and she's completely healed!

I was definitely more excited than she was... ever since it came off, she's been holding her arm and whining, "More purple!" I guess it can take a little while for her to get used to "not" having it :)

She's still favoring her right arm quite a bit, so it will take time for her to get used to using it again and to rebuild some of the lost strength.

But so far, no sucking her fingers! Fingers crossed that it continues...

I am SO happy that the cast is gone... it will make our lives just a little bit easier :)

Anyway, here are some pictures of the process:

Detours...(by Michael)

2011-12-09T20:43:00.003-06:00

As you may have read, the insurance company approved her power wheelchair. They approved it without blinking. That's really incredible considering that many people have to fight the insurance companies to get what their kids need. The feelings that filled our house when we knew she would have the wheelchair settled in for the night. Many of you celebrated with us through your words and thoughts.

As time slowly rolled by today I found myself thinking more and more about the Permobil K-450 that would soon arrive at our doorstep. The practical side of me is still excited. I'm excited for Ella to have a way to interact with her environment; excited to teach her how to use the 300+ lb piece of modern technological equipment that is a far cry from the kinds of wheelchairs available to kids who needed them when I was little.

Then my mind takes a detour as it so often has these past few months. A detour that I travel blindly. Travel it I must though, for it is part of what a parent of a child with a terminal disease must endure. A highway with no road signs, no speed limit, and no warning of the detour just ahead.

Someone spoke to me today...I literally did not hear them. They were speaking directly to me. My mind and senses were on a detour; this one was traveled on automatic pilot~I had no control of the thoughts that entered into the crevices of my mind. The reality of where I was became lost in the depths of the detour. It whisked me downward, almost as if I were spinning, and left me alone in a cold, damp, dwindling state of mind. The voice that beckoned for my attention was now silent and I felt several pairs of eyes upon me. The patient looks of those surrounding me gently lifted me from the detour and brought me back; quickly, smoothly and without judgement.

For the rest of the day and to the moment of writing this blog, the remnants of that detour remains.

I spent time with each of my kids this evening. Henry sat on my lap as we watched the Sesame Street character, Ernie, singing in the tub to his rubber ducky. I lay next to Ava before she fell asleep reassuring her that tomorrow is another day and she can start all over (she had a rough night!). I sat with Ella just before dinner watching her play with Play-Doh; organizing it, manipulating it, and generally focusing all of her attention on the task as hand.

I sat down to write this blog and while doing so received an email notification for a comment that was submitted on our last blog.

I read it. I stopped writing. I put the kids to bed.

Apparently, the person who makes the wheelchairs went in to work today (his day off, mind you) to make Ella's chair...just for her.

Another detour.

But this detour does not have the same markings as the other one I experienced today. This one quickly took me down a road of compassion and goodwill. The crevices that were previously filled with the darkness of SMA were now filling with the brightness of compassion. Ella is a fortunate girl.

I still hold fast to the fact that this wheelchair is good. I also am realistic with myself and know that I will struggle with the implications that go with it.

I know that the detours that SMA carries me through in my mind will continue; yet I also know that these detours hold the sum of all the experiences I will feel...the good, the bad, and everything in between.

Permobil K450 MX (by Lindsay)

2011-12-08T14:11:00.001-06:00

Oh my.

We got a call from the pediatrician's office today.

The insurance company approved the power wheelchair for Ella.

100% covered (except we have to pay the tax).

Wow.

I am so excited, I can hardly contain myself! Once Ella gets this chair (and masters it), she will be ALL over the place!

She's getting the Permobil K450 MX.

It will be SO good for her! The seat raises up and down, it tilts back for when she gets tired, and best of all, the chair lowers to the ground so she can interact with her peers! It will be so wonderful to have her be able to get around so much more independently outside of our house!

So now we're waiting for the vendor, National Seating and Mobility, to receive the official authorization from the insurance company. Then they'll place the order.

The vendor representative that we've been working with said we'll probably have the chair by the end of the year (like... in a few WEEKS!).

How incredible!

But now we really have our work cut out for us. We have to get a lift installed in our car. We have to get a ramp for our house. We'll have to do some rearranging in our house to try to make it more open for her, if possible. We've discussed putting something along the walls to prevent her from scratching and denting them while she's learning how to "drive".

We've talked about how getting the power wheelchair will be a turning point in our life. Life will be very different for us with this chair (and it will be quite the adjustment).

But it will be so worth it for our Squishy :)

Holiday Cheer (by Lindsay)

2011-12-05T15:42:00.020-06:00

Everywhere I turn, I'm reminded that the Christmas season is upon us.

But this year, it's different.

I'm not having much Christmas cheer.

Ask anyone who knows me pretty well and they'll tell you that my favorite time of year is between Thanksgiving and New Year's. It's just always such a magical time for me.

Well, it used to be.

This year, I didn't really want to get our Christmas tree. I didn't want to put up the decorations. To me, it was all just another thing to have to do.

A chore.

I was hoping that as the time drew nearer, my feelings would change.

But they didn't.

I see and hear people talking about their Christmas shopping. I haven't started. I not only haven't started, I haven't even BEGUN to think about it. I still feel like it's early fall, not December.

I kind of wish I could skip Christmas this year. Not the "true meaning" part, just the materialistic part.

I don't want to buy gifts. I don't want to receive gifts. It's all just "stuff" to me now. And quite frankly, we already have way too much stuff taking up too much space in our already crowded house. Space that we desperately need so Ella can get around more easily.

I don't mean to be such a downer. Believe me, I'm TRYING to have some holiday cheer. Especially for the kids.

But my heart just isn't in it yet.

There's only one thing I want for Christmas this year, and it's not something I can have.

A Matter of Time...(by Michael & Lindsay)

2011-12-03T09:15:00.007-06:00

We knew this would happen.

It was only just a matter of time.

The first time is the hardest.

We survived.

The Stomach Flu.

4:00 pm on a Sunday afternoon Henry launches his lunch and more on our couch. He spends the next six hours continuing in the same fashion. It's his first time being sick with the dreaded stomach flu and he lets us know in no uncertain terms that he is not a happy camper.

By 10:30 he has fallen asleep and wakes up every hour or so begging for water. We know that water will only make him sick again so we wait as long as possible before we give some (a very little, mind you)...he holds it down.

Lindsay does 10 loads of laundry between couch covers and cushions, clothes, blankets, pillow cases, and towels (and pretty much anything else that would fit in the washing machine).

Ava and Ella have escaped the fates that the stomach flu hold.

Until...

Tuesday night at 10:30, Ava launches her version of the stomach flu on her bed (in a room she shares with Ella). We gather Ava up, bathe her, and set up the small mattress next to our bed. Her eight-hour stint begins yet she is more aggressive with her projectiles than Henry was (Ava is a bit more experienced in this area)...she takes her eight hours and uses the sick bowl every 10 minutes (on average). Lindsay and I get absolutely no sleep...none...nada.

Wednesday rolls slowly around. Lindsay continues to do laundry, disinfect everything in sight, and generally sterilizes the house.

Ella has a doctor appointment on Wednesday. Dr. Murphy, our beloved pediatrician, supplies Lindsay with anti-nausea medication in case Ella decides to follow in her siblings' recent examples. We pray she doesn't.

Wednesday night rolls around and we put all three kids to bed. We are exhausted and get to bed as early as we can.

All is quiet.

Ella whimpers at 2:30 am. I am awake (as usual) and I try mental telepathy to her to go back asleep (I swear that works sometimes). Her cry gets a bit more intense. I get up, whipping the covers off. Ava, by the way, is still sleeping in our room as we wanted to keep her away from Ella one more night.

I rush into Ella's room and catch a whiff of the inescapable result of the stomach flu. It's dark but the moonlight casts a faint glow on Ella's head. The smell is stronger. I hear her gurgle and as I approach her crib I see the stomach flu doing it's nasty work.

Yet this time it's different.

Ella cannot move enough to expel the contents of her stomach away from her body...she could very easily choke. I place my hand on the side of her head and turn it for her as she lets out what she had eaten that day. I have to wait until she's done before I can pick her up since I know she won't help me as I scoop her up. I call for Lindsay and she's by my side in an instant.

When the episode is finished I cup one hand behind her neck and the other under her rump and scoop her up in one swoop. I place her on the floor in a sitting position...she is wobbly at best and confused, her little hands shaking more than usual and her eyes asking, "What's happening?". She has more to do and we have a small container available that we use to catch her contents. Supporting a child with SMA as they vomit is so much different that supporting a person who has full function of their muscular system.

Lindsay doesn't miss a beat as she retrieves the medication that Dr. Murphy had given her earlier; we quickly measure out the dosage and Ella takes it trustingly.

We bathe our Squishy.

She didn't have another episode the entire night. She fell asleep after a while. We, of course, did not sleep because we wanted to make sure she didn't get sick again and choke. Night #2 of no sleep.

The toll that would have been taken on her already fragile body if she had to endure the typical six to eight hours of fighting the stomach flu was averted. I couldn't imagine what kind of repercussions would have followed.

Lindsay continued to clean, clean, clean.

We made it through our first real sickness with our Squishy. We know there will be more times when she gets sick, but it is good to know we have Dr. Murphy and her staff looking out for us in so many ways, and that Lindsay and I can work together, despite the gross lack of sleep, to do our best to take care of our children.