Done with Diapers!! (by Lindsay)

It has been a long Spring Break.  Very looooooooong.

This potty training thing has been one of the most challenging things I've ever done as a parent.  It is SO much harder potty training a child with SMA than it is a child without.  It is physically and emotionally exhausting.

We started last Friday, and luckily Ella quickly learned how to pee in the potty.  Unfortunately, being on an all-liquid diet, she needed to go about every 10-15 minutes.  If she was sitting on the potty, she'd go there.  If she was somewhere else, she'd go wherever she was.  She had no idea how to hold it or tell us if she needed to go.

After almost two full days of this, I was about ready to give up.  It was too hard and too stressful.  But I forced myself to push through, knowing that I had given myself all of Spring Break for this process.

Luckily, by the end of the day on Saturday (the second day), she started holding it longer and even sometimes telling us that she needed to go.  From that point on, she never had a pee accident again :)

And then there's the poop.

Oh, the poop.

She basically decided that she didn't want to poop in the potty.  Ever.  Every day, she would go in her underwear 3 to 5 times.  Every single day, for seven days in a row.  We'd ask her if she needed to go poop (knowing her basic "poop schedule"), she'd say "Nope," and then go in her underwear within 30 seconds.  Almost every time.  And I think she secretly loved it.  Grrrr...

We bribed her with toys, candy, her iPad, walks outside, etc. but NOTHING worked.  We tried getting angry with her.  No good.  We tried being indifferent about it.  Still nothing.

Finally on Thursday, after she pooped three times in her pants during her clinic appointment, we decided to increase her Miralax dosage that goes into her daily formula (with her doctor's approval, of course). We actually tripled it :)  She normally has a small dose of Miralax every day to help keep her stools soft so that it's not hard for her to push.  But by increasing her dose that much, it made it so soft that she "accidentally" went poop on the potty the next day.  

And I think that's what did it.  She realized that it was ok to go in the potty.  That first day after the Miralax increase (yesterday) she still had a few small accidents in her underwear, but she also went poop in the potty a few times. 

And today (Saturday), she didn't have a SINGLE accident ALL DAY!!  She even stays dry during her naps!  We are thrilled!!  She really gets it now.  And she is SO proud of herself :)  

So tonight, Ella got her reward.  We took her to Build-A-Bear to pick out a new friend :)  She was so excited and Ava and Henry were equally excited for her!  

It has been a lot of work this past week, but totally worth it!  However, now I feel like I need a "real" Spring Break, ha ha :)

Such a big girl!

Ella with the doggie she picked out (she named him "Trevor").

Kissing the heart that goes inside

Ella and Trevor in his doctor outfit :)

We even got a wheelchair for Trevor!

Through It All...by Michael & Lindsay

Ellen (resident), Linda (Ella's PT), Ella, and Mary Massery

The effects of SMA reach far beyond what the eye can see.  As Ella grows, so do the complications associated with the disease that ravishes her muscles, taxes her patience, and elicits questions that no child should have to ask.

About 6 months ago (October, 2012) we had a routine check-up with Dr. Prestridge, Ella's Pulmonary Specialist.  We spoke mainly about general respiratory health and bipap settings.  Of course a physical examination of her chest ensued.  One thing the doctor noticed was a small protrusion at about the center of Ella's chest.  She said this was typical for people with weakened muscles.  She then proceeded to refer us to a highly recommended physical therapist who specializes in pulmonary/respiratory PT.

Her name is Mary Massery.

Mary Massery has a vast wealth of experience and expertise in terms of pulmonary/respiratory health.  She has been in practice for 20+ years.  When we called for an appointment the first available was over 3 months out.  They couldn't even schedule us.  We were told to call back in two months.  We did.  The scheduling was available 4 months out and the date of March 20, 2013 was available...we took it.  A total wait time of 6 months to see Mary.  In addition, her services are not covered by insurance...the cost was well worth it, however.

The appointment lasted a total of two-hours.  We sat with Mary and had a wonderful conversation about Ella.  Her complete history, her strengths, her weaknesses, our hopes, and our questions.  Mary spoke with us with grace and compassion, knowledge and sensitivity.

She made recommendation for feeding schedules which we will discuss with Ella's SMA Team at Lurie's Children's Hospital.  She gave advice about travelling, the SMA Conference, the general health and well-being of a person with a neuromuscular disease.

Of course, the physical exam came next.  We were a bit apprehensive about the protrusion that Ella has developed.  Mary, once Ella was down to her diaper, took one look at the protrusion and smiled.  She said it looked "great".  Great??  What does that mean? I thought.

Apparently the protrusions such as this one can occur one of two ways.  The chest cavity can expand out or sink in.  If one were to have a protrusion one would want to have the kind that expands out.  It allows for more breathing room and indicates that the chest muscles are not as weak as one with a sinking protrusion.  Ella has the expanded type protrusion.  The size of the protrusion is also a factor she looked for.  She was absolutely not concerned at all with the size of Ella's.

She performed many manual tests on Ella's chest, ribs, diaphragm, and back.  She took measurements, she drew lines on her body, she took pictures, she laughed and giggled, tickled and played with her.  They had a grand 'ole time.  In the end, she was very pleased with Ella in terms of her pulmonary/respiratory function given the disease she has.

She was kind enough to also show us how to manually "cough-assist" Ella if need be.  It was kind of weird doing the procedure because in doing so, you can feel her ribs and chest cavity.  The natural thought is that one might "break' something in there...but the more I did it and from watching Ella's reaction, the concerns quickly fled (see video).

All in all, visiting Mary was extremely beneficial to us as Ella's parents.  We learned much about the pulmonary/respiratory care Ella needs and will need, we walked away with more ways to help her, and we feel more confident in working with her on a daily basis.

Yes, SMA presents challenges we never dreamed a child could go through, let alone our own...but there are people out there who work so hard to help so many.  As this disease progresses, and it will, our job as her parents is to love her, care for her, and be with her...through it all.

It's Potty Time! (by Lindsay)

Going to the bathroom.

It's not something that you and I give much thought to throughout the day.

But Ella does.  She has wanted to use the potty and wear "big girl" underwear for months now.

We potty trained both Ava and Henry right when they turned two years old.  One day they wore diapers, and the next they wore underwear. Cold turkey. And it was actually quite easy.  Of course there were some accidents in the beginning, but within a few days, they had it down.

With Ella it's a little different.

Once she's trained, the steps involved with each trip to the bathroom will look a little different compared to the "typical" toddler:

1. She'll tell us she needs to go potty.
2. We'll take her to a comfortable spot on the floor and lay her down.
3. We may or may not need to remove her TLSO (back brace)...currently she wears leggings that go under the brace, but hopefully we'll make it work so they go over it.
4. We'll remove her pants and underwear.
5. We'll carry her to the nearest toilet and place her on it.
6. We'll sit with her so she doesn't fall.
7. We'll wipe for her.
8. We'll flush for her.
9. We'll help her wash her hands.
10. We'll carry her back to the floor and lay her down again.
11. We'll put her underwear and pants back on (and possibly her TLSO).
12. We'll carry her back to wherever she was before she went to the bathroom.

Phew!

And the process will be like this. Every time. Always.

Michael and I made the decision not to potty train Ella at two years old like her siblings. We decided we wanted (and maybe needed) to prolong the convenience of diapers a little longer.  We knew that once Ella is potty trained, we will always have to drop everything (every time) when she needs to go to the bathroom.  Unlike Ava and Henry, there won't be a time when she can go to the bathroom by herself.

But over the past six months or so, Ella has been asking.  And asking.  And asking to use the potty by herself. Occasionally we've put her on the potty and she just enjoys sitting there, but she never does anything other than sit. But she continues to ask to use the potty and wear underwear.

So around Christmas time, Michael and I set the date for potty training...Spring Break. We knew it would be a good time since both of us would be home with the kids and we'd have a solid week to work on it with her.

Our Spring Break begins this Friday. And Ella couldn't be more excited!  I don't quite share the same level of enthusiasm that she has since having Ella potty trained will add a new challenge to our life.  But as with all of the other challenges and changes that we've had since Ella's diagnosis, I'm sure we'll get used to it in no time :)

And I am SO ready to be done with diapers!  For GOOD!!

This is the potty seat made by Bumbo that was recommended by another SMA parent...it provides a lot more support than other potty seats!

If SMA Had Its Way...(by Michael)

Birthday parties are awesome, they are so much fun for kids.  A time to play, eat, dance, socialize, and celebrate.  We were invited to a friend of Henry's birthday party and the invitation was graciously extended to Ava and Ella as well.

We knew there would be a moon-bounce at this party.  We knew that Ava and Henry would absolutely love playing in it, as most kids do.  We also knew that Ella would most likely want to join in the fun.  If SMA had its way, she would not be able to enjoy the jumping fun that every child in the room was enjoying.  If SMA had its way, she would sit on the sidelines with wide eyes, wishing she could join the others.  If SMA had its way she would be idle as the children she looked upon felt the exhilaration of bouncing, flipping, and soaring through the air.  If SMA had its way, she would not be able to enjoy the simple pleasure of childhood.

She was persistent in her desire to go into the moon-bounce.  Lindsay and I didn't have to think about it too much in terms of letting her go...we did have to think about how to best hold her so as to give her the best experience she could have.  SMA wasn't going to take this away from her.

I was the first one to go with her into the bouncy-heaven. Needless to say, it took considerable effort to hold my own in the "arena" and even more so to keep her body in a comfortable and safe position.  We experimented quickly with a few different positions and quickly found a comfort zone.

Her smile was priceless.  Her excitement was contagious.  

We bounced together.  We sat and felt our bodies rocking to the beat of those around us.  She got to feel the anti-gravity that so often eludes her.

After a break it was Lindsay's turn to enter the childhood pleasures with Ella.  They laughed and played.  They forgot for a moment the awful disease that afflicts Ella.  They enjoyed the moment for what it was...a childhood moment.

If SMA had its way then Ella would not experience much of the world.  Through her determination, her will, and her love of what life does have to offer, she defies SMA every chance she gets.

Take that SMA.

"Is that MY school??" (by Lindsay)

Today Ella had her evaluation at Ann Reid Early Childhood Center, where she will be attending preschool in the fall.

To say she was excited would be an understatement.  Ella has been looking forward to going to school for such a long time. When we pulled into the parking lot, her face immediately lit up while she said, "Is that MY school??"

While Ella was being evaluated, Michael and I were interviewed by various members of the team, so that they were able to learn more about Ella as well as any of our concerns.

This was our second meeting at Ann Reid, and we were once again very impressed. All of the team members seemed very knowledgable of the needs of a child with a neuromuscular disease (the school has actually had children with SMA before)! Among those at the meeting were the assistant principal, school nurse, social worker, psychologist, speech pathologist, physical therapist and occupational therapist.

Ella was also taken on a tour of the building, where she got to see some classrooms and even met a little boy who is also in a wheelchair :)

The Ann Reid Early Childhood Center is only a couple years old.  This school will be perfect for Ella.  Everything is state-of-the-art and will make things easier for Ella.  From the standers and gait trainers, to the small sinks with automatic soap dispensers, Ella will have an easier time doing more things independently, which is one of the main goals that the team has for her.

Ella will be in a "blended" classroom.  In this type of classroom, half of the students have special needs and the other half does not (aka "community students").  There is one teacher and two aids in every blended classroom of 15 students. There is the possibility that Ella will also have her own nurse, depending on whether or not she needs to do a feeding tube feed while at school.

Speaking of community students, Michael and I made the difficult decision to enroll Henry at Ann Reid for his second year of preschool next year. This year, Henry is attending Sky Blue Scholars (where Ava attended as well), and we absolutely LOVE it. Sky Blue has become like a family to us and we are so impressed with their educational philosophies. It was a hard decision, but in the end we decided that it was a better fit for our family to have Henry and Ella attend the same school (rather than having three kids at three different schools).

As of now, the plan is to have Henry and Ella take the bus to school together, and they couldn't be more thrilled :) Ella will attend Monday though Friday mornings and Henry will attend Monday through Thursday mornings (only children with special needs go on Fridays). With Ava being in 1st grade, next year is going to be quite a transition for me, having four mornings every week with no children around! It will be a whole new life of running errands and doing housework alone...and who knows, maybe I'll have a little time for "me" :)

One thing we're a little apprehensive about is Ella's expectations, because so far, she thinks that when she goes to preschool she'll be able stand and walk by herself :( She still thinks that her inability to do such things is a result of her age...she doesn't yet understand the harsh realities of SMA. But we're hoping that our visit today helped a little bit, by showing her that there are other kids at school in wheelchairs, standers, etc. Plus, with the guidance of Ella's psychologist, we've gradually been having more conversations with Ella about going to school in her wheelchair.

But we're definitely really looking forward to Ella going to Ann Reid. We think it's going to be such a good fit for her, both physically and emotionally, and we can't wait to see that bright mind of hers put to work!!

Burlap & Cotton...(by Michael)

Imagine a burlap bag.
It's being filled with cotton.
The cotton is soft, it's light, it's squishy.

Imagine more and more cotton being placed into the burlap bag.  The incoming cotton seems to meld with the existing cotton already in the bag.  The "old' and "new" cotton become one, each allowing the other to coexist.  

Now imagine the burlap bag, with the cotton inside of it, beginning to fill to capacity.  More cotton is introduced and it becomes more difficult to fit in the "new" cotton.  The burlap bag begins to stretch to accommodate this influx of cotton.  The cotton itself becomes pressed for space and is losing its softness, changing rather, to a hard clump in an effort to exist.  More cotton is being added and the burlap bag begins to stretch to its ultimate limit.  The cotton is no longer soft and fluffy...it is hard and unyielding.  More cotton is being stuffed, forced into the bag.  The seams of the burlap bag begin to stretch, desperately trying to hold itself together under the pressure of the cotton within. Day after day, hour after hour, minute after minute...

Sometimes there is a reprieve and some of the cotton is allowed to escape, providing a temporary respite for the burlap bag.  Of course, more cotton awaits and will find its way into the bag. 

What will become of the burlap bag?  When, if ever, will the cotton stop being jammed relentlessly into it?  


The pressures that surround our lives often feel like the scenario of the burlap bag and the cotton.  We find ourselves in the midst of perpetual stress.  The demands placed upon us have become commonplace; part of our daily lives; nonetheless they tax our reserves of patience, compassion, and sometimes good judgement.  We look to each other for strength knowing that the other may not have it readily available at that moment.  

Caring for a person whose muscles are slowly fading away has proven to be exhausting...physically, mentally, socially, and spiritually.

Knowing that, unless the medical community finds treatments or a cure for SMA, we will spend the rest of Ella's life providing not only what parents normally provide for their children, but also providing her a means to manipulate the world and her movements as well.  

Wondering what will happen, how to get through one more day of exhaustion, and trying to reconcile within our own minds any guilt, lack of control, or reasons for the plight that has been given to our daughter saps mental energy quickly.  The mind wanders, dreams lurk, reality begs an answer.

Seeking time with others, friends and family, brings with it a logistic puzzle.  We are fortunate to be surrounded by people who are sensitive to Ella's needs; helping in so many ways.  Ella loves to visit people and go places which tells us that those around her care deeply about her well-being.

Before Ella was born we were led to a church community that has brought comfort, support and friendships.  The love and kindness shown by those who surround us brings meaning to our lives in untold ways.  We have found ways to seek the guidance God provides and to allow the words of Christ to breathe the breath of life into us.  While we do not understand the ways of the universe, or the circumstances that surround us, or even the reasons why they exist, we are thankful for the opportunity to spiritually grow within the context of the life we have been gifted.  To shake a fist at the Almighty may be something we engage in, yet we know that He loves us and our dear Ella, Ava, and Henry.

So our burlap bag seems to be perpetually full...bringing the seams therein to the point of last resistance. We go on, each and every day...we know not how.  We go on, day in and day out, despite the challenges that SMA brings to Ella.  

We go on...physically, mentally, socially, and spiritually; with a deep desire to care for our children.