A New Chapter...(by Michael)

For years I've taken Ella to her therapies.  Each time I sit and watch.  I watch and wonder why SMA has to exist; why do people have to have such horrible diseases.  My mind becomes occupied with these thoughts so I divert my attention by doing Facebook, catching up on emails, or grading papers.

It's been just over 5 years since Ella's diagnosis.  I can remember that day clearly.  After crying, Lindsay and I sat on the floor in front of our stairs (in our old house) with Ella playing innocently in front of us.  We looked at her, not knowing what the future would hold for our family.  We made that promise to each other, the one about not being able to save her but being able to care for her until they find a treatment or cure.

I sat, on December 23, 2016, watching Ella work while she was at her therapy session, as I always have.  I decided to check Facebook and the first post that appeared was a shared post from the Cure SMA Organization..."Breaking News.  FDA Approves Spinraza (Nusinersen) for SMA".

Wait.

What??

Yes.  I read that right...and so did you!!  The FDA has finally approved a viable treatment for SMA. This is huge; this is unbelievable; this is the best Christmas present any SMA family could imagine. A treatment.  Something for us to hold on to; something for us to keep our hopes alive.  Something for our kids.

The drug targets the back up gene that produces the missing SMN (Survival of Motor Neuron) protein.  The back-up gene however has what they call a "splicing error" and therefore only produces roughly 10% of a functional SMN protein. Kids can have multiple back-up copies which helps determine the severity of their SMA.  Ella has 3 back-up copies; therefore she produces about 30% of the functional SMN protein needed to keep her motor neurons alive.

This drug targets those back-up copies and directs the mechanism which produces the SMN protein to NOT make the splicing error...in turn, more functional proteins are made.  The drug has to be administered into the spinal column throughout the course of a person's life to maintain effectiveness.  Small price to pay, I'd say.

This is game changer.  For infants newly diagnosed it can mean wondrous things for them.  For Ella, it means potentially slowing down the progression of the disease...a wondrous thing in and of itself.

The drug is due to hit the marketplace in early 2017 and the SMA Community can hardly wait.  The whole community is buzzing all over the internet about this incredible news.

So many of you have followed our story, prayed for our family as well as those stricken with this disease, and supported us in so many ways.  We know that you, too, have held hope for a breakthrough such as this...and incredibly so...it is actually here.

 A new chapter in our story will unfold before your eyes.

Click here to read the Cure SMA article!

Discouraged (by Lindsay)

Our house is turning into a hospital. 

We have medical supplies in almost every room.

We've been on a bit of a "recovery roller-coaster" since I was discharged from the hospital a week and a half ago. 

I was discharged with a portable Wound Vac for home use (24/7) and I have a home-care nurse who comes every Monday, Wednesday and Friday to change the dressing and evaluate the incision. 

My portable Wound Vac

It's nice to have a carrying case for the Wound Vac!

I was also sent home taking the oral antibiotic, Ciprofloxacin. Unfortunately, after a few days, I began to experience very negative side effects from the medication...extreme fatigue, nausea and horrible dizziness. 

By Saturday (11/12), the side effects had gotten so bad that I had to call my infectious disease doctor, and he instructed me to stop taking the Cipro for the rest of the weekend to see if I felt better.

I felt MUCH better :)

So Monday morning he prescribed Levofloxacin, which is very similar to Cipro, but some people have an easier time tolerating one versus the other. Unfortunately, for me, the side effects were the same as the Cipro, only this time I was vomiting as well. 

I called my doctor that evening and he told me it was time to have a PICC line put in so that I could receive IV antibiotics at home.

He ordered the PICC line on Tuesday, however, our insurance company took quite a while to approve it. Because I had gone a while without antibiotics (and we still hadn't heard from our insurance), on Wednesday evening, my doctor had me go to the ER to receive a dose of the IV antibiotics. Then I had to go again on Thursday evening (ER visits are not fun and not cheap).

IV antibiotics in the ER :(

Finally on Friday, we got the approval from our insurance. I had the PICC line placed this morning (Saturday), so now I will be receiving my new IV antibiotics at home!

I am taking Cephtriaxone for six weeks and then we will reevaluate. I get one dose every 24 hours (which I am learning how to administer myself!) and then I'll have the dressing on my PICC line changed every week (this is a sterile process, so it has to be done by a nurse). I already have my wound vac dressing changed three times per week, so my nurse will also change the PICC line dressing during one of those visits. 

New PICC line

Everything that goes into the PICC line for each daily dose of antibiotics

Getting ready for my first dose!

One step closer to getting my M.D. :)

To protect the PICC line when it's not in use, the nurse suggested cutting a tube sock to cover it :) I've already ordered myself some knee-high Christmas socks to cut and start using the day after Thanksgiving ;)

Our dining room has become my "hospital room"

We are feeling so relieved now that I'm receiving the IV antibiotics...it had been a very stressful week knowing that I wasn't getting the medicine to help fight this infection and prevent it from spreading to the hardware or my bones.

I saw my orthopedic surgeon this past Thursday, and overall he was happy with how my incision looks, especially considering how long I had gone without antibiotics. He's having me continue to use the Wound Vac 24/7 for another two weeks and he will reevaluate the incision at that point. He's hopeful that it should be healed enough to remove the stitches then. He did, however, instruct me to no longer bear any weight (even on my heel) for the next two weeks. I've had a significant increase in pain this past week, but luckily it's become much more tolerable since I was told to stop walking on it (as inconvenient as it is). 

However, our moods went a bit downhill yesterday. 

When my nurse came for my Wound Vac dressing change, we unfortunately found that another hole is developing along the incision, with infected tissue at the bottom. This hole was not there 24 hours earlier. We're confused about why it's there...I had already received two doses of the IV antibiotics and I had been non-weight bearing since we last looked at the incision (when the hole wasn't there). We know that the healing process can take a long time, but it's not typical for the incision to get worse. And we also know that things can develop/change extremely fast with this type of infection.

My nurse was concerned. She called both my orthopedic surgeon and infectious disease doctor to let them know. We're really hoping that my incision will improve by keeping the Wound Vac in place and consistently administering the IV antibiotics. We won't get to see the incision again until my next dressing change on Monday. If there is no improvement, I will need to have it looked at by one of my doctors (currently my next appointments are scheduled for the week after Thanksgiving).

Our emotions have been ALL over the place for the past few weeks. One minute we think we have things under control and are headed in a positive direction, and then we receive more concerning news. 

We're discouraged.

This is no longer about the inconvenience of recovering from four surgeries. For the first time, this past week I broke down with the thought of, "What if..."

We're scared. 

My Foot - Surgery #4!! (By Lindsay)

Well...

A lot has happened since my post last Friday about the infection in my foot.

It continued to get worse and by Saturday I ended up in the emergency room at Edward Hospital because of a hole that had developed in my foot.

The ER doctors were concerned that the infection had possibly spread to the hardware in my foot and/or bones. They called my orthopedic surgeon (Dr. Arndt) and I was admitted to the hospital and started IV antibiotics immediately. They scheduled me to have surgery #4 the next morning (Sunday) to try to get rid of the infected tissue and to see if it had spread (the surgery was performed by Dr. Arndt).

The surgery went well, and there were no initial signs that the infection had spread to the hardware or my bones - yay! Dr. Arndt successfully removed all of the dead and infected tissues, and took many cultures to determine which infection(s) were present. All but one culture has come back, and so far I've tested positive for E. coli (not the type that's common with the digestive tract, but a kind that can sometimes occur in an incision after a surgery). The team immediately switched my IV antibiotic to one that is geared toward this type of E. coli. 

They placed a "wound vac" in place, which essentially is a foam pad over the incision with a tube that connects it to a vacuum-type machine. This machine virtually sucks out any drainage as it develops, helping to prevent another infection from developing and speeding up the healing process. I will be using the wound vac at home 24/7 for the next two to three weeks, having a home care nurse coming three times per week to change the foam pad dressing. 

I had my first dressing change in the hospital today, and was pleasantly surprised to see that most of the incision had been able to be closed with stitches! We were told by the wound care team that the foam pad is typically placed inside the open wound and changing it is very painful. But since I have the stitches in place, the wound is almost completely closed, so the dressing change was a piece of cake! They will continue with the wound vac, as there still has been drainage, and we need to be certain that it gets sucked away from the incision. 

I was discharged from the hospital this afternoon (Tuesday)! However, I was told that this next week is critical to ensure that a new infection doesn't develop, as the tissue in that area is very fragile. I was sent home with an oral version of the antibiotic for this strain of E. coli, which I will probably be taking for several months. Having so much hardware in my foot puts me at a higher risk of an infection developing and/or spreading in that area. 

My instructions for the next week basically have me elevating my foot as much as possible. The wound care team said that I need to avoid any swelling as much as possible, as that is basically drainage inside the wound that can cause another infection. I can still be up and around briefly using the crutches or knee scooter, but only when I really have to be. 

In one week I will go back to Dr. Arndt to check my progress and see where to go from there. Fortunately, from an orthopedic standpoint, nothing has changed in terms of my recovery from surgery #3 six weeks ago since my bones and the hardware were not affected by this infection. I will also be needing to go see the infectious disease doctor in a couple weeks so that he can assess the wound and take cultures to be sure that I continue to be infection-free. 

We cannot even begin to thank all of you for your kind words of support, thoughts and prayers during all of this! And especially to those who have been helping out with our family while I've been in the hospital for the past four days (and of course those who've called, texted, emailed and visited me)!!

Many of you have asked how you can help (we don't even know what to say...we have been SO blessed by our very LARGE supportive community over the years with Ella's diagnosis & surgeries, and now my surgeries in the past year!). 

Right now we're still trying to figure out what our needs will be, as this infection/surgery was unexpected and we're not sure exactly what my recovery will entail (there may be last minute appointments, setbacks, etc.). A lot of people have asked about starting a meal train, and we definitely feel that dinners will be extremely helpful right now! However, with the uncertainty of what each day will bring, it's hard for us to commit to any type of schedule for people to sign up to bring a meal. For those families who have asked to bring a meal, we are wondering if it would be possible for a couple families to go in together on a restaurant gift card for a place that either delivers or has carry out. We've learned from Ella's past surgeries that this provides a lot of flexibility for us when we aren't 100% certain of what each day will bring. 

We're not sure yet if we'll be needing an extra set of hands on weekdays once the kids get home from school before Michael gets home from work. We will have to take it one day at a time and go from there. 

Again, we can't thank everyone enough for the love and support you have given to our family. We are so incredibly comforted knowing we are not alone during all of this!!

My Foot (by Lindsay)

An update on my right foot...

The surgery that I had back in September was a success! My orthopedic surgeon was able to cut and remove the part of the bone that was causing the added stress to the other joints of my foot. He inserted a new plate as well as seven screws to hold everything in place, which will all be permanent. My bones have not yet started to fuse together, but they will as it heals.

I was non-weight bearing for about the first four weeks after the surgery (a little longer than expected, but my doctor wants to be sure that this surgery AND recovery are done correctly). By about third week of being off my foot, I was basically pain free!

Two weeks ago, my stitches were removed and I was given permission to start walking on my heel with the boot cast on. Of course I was very excited, as a whole new world had been re-opened to me! It's amazing what we take for granted!

A couple days after that appointment, I started developing some pain in my foot, which I assumed was from the new weight-bearing. 

Then it got worse.

By the following Monday, I could hardly handle being in a vertical position, let alone put ANY weight on my foot. I called my orthopedic surgeon and we discussed my incision and the area surrounding it.

The incision was a mess. It was constantly oozing and had an odor. There was redness around the incision, which was hot to the touch and spreading. My entire foot was starting to swell. 

Infection.

He immediately put me on antibiotics and within a few days I was able to put a little weight on my heel again (although it was still pretty painful). 

10 days later I finished the antibiotics and I had another routine follow-up with my doctor (yesterday). While my foot does look better than it was, he was not happy with how deep the infection has gone into my foot. The incision still looks pretty bad and I'm also in a fair amount of pain. And I've been having off-and-on fevers since the infection first started.

He started me on another round of antibiotics and is having me do a "wet to dry" dressing every day (a gauze pad soaked in sterile saline solution placed on the incision, followed by a dry gauze pad, and then covered with an ace bandage wrap around my foot/ankle to hold it in place). I am also to stay off my foot again and elevate it as much as possible. 

Wet to Dry dressing materials

I'm going back to see him in a week (next Thursday) to reevaluate. 

If the infection has not shown significant improvement, he said we'll need to go back to the operating room to address the issue. 

Ugh. 

Surgery Tomorrow (by Lindsay)

Tomorrow morning I'm going in to Edward Hospital at 5:30 am to have surgery on my right foot.

None of us are looking forward to it, but we ARE looking forward to being done with foot surgeries! Fingers crossed that the 3rd time's the charm!

We've been doing a lot to set up life at home for the next 5 weeks, as I'll be unable to walk without crutches, drive, or carry Ella. 

Our night nurse has changed her shift from 10 pm - 6 am to 11 pm - 7 am so that she can get Ella up and dressed in the mornings.

Our incredible school (and school district) is allowing Ella, Ava and Henry to ride the bus to and from school every day until I'm able to walk them! This was pretty amazing, since we're "walkers" so they would not normally allow Ava and Henry to join Ella (Ella DOES qualify for the bus because of her SMA, but since we live so close and are walking anyway, we've always had her walk with us). But this will relieve A LOT of stress about having the kids walk home alone or in bad weather! Thank you, Prairie Elementary and Naperville District 203!!!

We then found out that Ella has over 200 hours of emergency/respite nursing care that expire at the end of October (and then we'll get another 350 hours for the next year), so we'll definitely be using them! As of now, we have a new nurse who will be coming to our house on Monday and Wednesdays after school for four hours (until I'm given clearance to carry Ella), so that Michael can continue to tutor those days after school. Our nursing agency is also looking for a possible part-time nurse to help on weekends, so that Michael is able to get things done around the house, yard, etc.

My parents will be able to help out on some Thursdays and Fridays (Ella has therapy those days after school), taking her to PT/OT and aqua therapy. Michael will be able to meet them there, stay with Ella, and then bring her home (so that my parents can leave after they drop her off).

Many people have asked how they can help (thank you!), and I think the only thing left would be to assist with a few dinners, so that Michael doesn't have to cook every night.

Like we have in the past with some of Ella's surgeries, we've set up a Care Calendar with days over the next month that we would love a meal (or restaurant gift card for somewhere that has delivery or carry-out). Here is the link and sign-in information for the calendar:

http://www.carecalendar.org/logon/243115

Calendar ID: 243115
Security Code: 1914

Of course there is NO pressure to bring a meal...we've just had several people ask if we are going to set up the Care Calendar for meals again :)

We're hoping and praying for a smooth surgery and quick recovery! Thank you all for your kind thoughts and prayers up to this point! We are so blessed to be a part of such an incredibly kind and supportive community!!!

My Foot (by Lindsay)

In the past year I have had two surgeries to correct pain I was having as a result of an old gymnastics injury.

The injury was at the ball of my right foot, so I was referred to a podiatrist. During the exam, he said he could easily fix that joint by shaving down that part of bone that had grown over when my injury healed years ago.

But I was also told that the main bone in my foot, leading to that joint, was too high and needed to be lowered.

It involved cutting the bone, lowering it, inserting synthetic bone as a bone graft, and finally placing a small plate with screws to hold it all together.

My initial X-ray taken "BEFORE" the first surgery last fall.

I was told I would be "good as new" in three to four months.

The recovery was a bit tough...I had to miss two weeks of work and I was in a boot cast for quite a while. We had to pay our babysitter to stay with our kids until Michael got home from school, since I was unable to lift Ella for those first couple weeks after the surgery.

After I was done wearing the boot, I was still in quite a bit of pain.

In mid-winter the podiatrist decided I needed custom orthotics to wear with gym shoes to give my arches added support.

That helped a little.

But I was starting to have pain on the top of my foot where the plate and screws were placed and in my ankle, and a hard bump was forming around where the plate was. Large enough that I had to buy several new pairs of shoes, as my foot would not fit into the ones I currently had.

So in the spring, the podiatrist said that the plate and screws were causing irritation to the main tendon that runs along the top of my foot, and since the bones had all healed from the surgery, I could have another "minor" surgery to have the plate and screws removed.

I decided to wait until school was out, so that I would not have to miss any more work.

When that surgery was performed in June, the podiatrist found that there was a lot of bone growth around the plate and screws (hence the hard bump that had formed), which all had to be scraped away. So the recovery was a bit longer and more intense than we had planned.

In July I went in for my final follow-up appointment with the podiatrist and he said that everything looks great and I can now resume full activity, wear any kind of shoes I'd like (with or without the orthotics), etc.

But I was still in pain.

He told me it would continue to get better in time.

But it didn't.

It was bothering me enough that I was walking with my weight on the outside of my foot since it hurt to put pressure in the middle of my foot. Walking like this was starting to cause knee and back pain, and it was becoming painful to lift and carry Ella.

This was unacceptable.

After talking with several people (including medical professionals), Michael and I decided that I should go see an orthopedic surgeon for a second opinion.

I was able to get in to see one who specializes in feet and ankles, as well as sports medicine.

After I explained to the orthopedic surgeon ALL that had happened in the past year and once he had looked at my (many) X-rays, he told me that this was a very interesting case.

He told me that my foot is now worse off than it was before the initial surgery last fall.

Super.

The part of the surgery that was done on the long bone of my foot (involving the cutting, bone graft and plates/screws) has lengthened that bone and caused a joint to be eliminated, which is causing tremendous (and painful) stress on the joint next to it. The joints in my other four bones are trying to bend in one place, while the joint in my first bone is trying to bend in another.

Below are three "After" x-rays, taken in July 2016, following the plate and screws removal.

The first "AFTER" x-ray taken in July 2016

The second "AFTER" x-ray taken in July 2016

The third "AFTER" x-ray taken in July 2016

Ouch.

So I'm now back in the boot.

And I have to have surgery #3 (which has been confirmed by several other medical professionals).

The orthopedic surgeon will be cutting out a piece of the bone where the joint was eliminated, shortening that bone to its original length (my right foot grew a size and a half since the first surgery...now I know why!), moving the remaining bone closer in to close the gap, fusing it to the bone it meets, and finally inserting a permanent plate with screws (on the side of the bone so that it does not irritate the tendon). There may also be additional screws needed to assist with the bone fusion.

It will be the most complex of the three surgeries. It will be outpatient at Edward Hospital (rather than a surgical center like the other two) and I will be having general anesthesia (I only had twilight for the others).

The recovery will also be longer and more difficult.

I will be in a half-cast splint lying down with my foot elevated for the first week after the surgery. He told me I will be in quite a bit of pain and will require some high-powered pain meds.

Then I will have the cast taken off and I will be back in the boot cast for 4 weeks using crutches. I can put some weight on the heel of my foot during this time.

After that, I will be able to walk with the boot cast, but will need to wear it for an additional 6 weeks.

So...

I will not be able to carry Ella (or walk the kids to and from school, walk the dog, etc.) for at least FIVE WEEKS after the surgery. This is where it get's tough.

Michael will have to leave later for work in the morning, so that he can get Ella up, dressed, take her to the bathroom and bring her downstairs (he's usually long gone before the kids wake up).

After school is a different story as our kids are done at 2:30 pm and Michael's students aren't dismissed until 3:00 pm (in Elmhurst). Plus Michael tutors a couple days per week and doesn't get home until much later those days.

Ella also has therapies on Thursdays and Fridays after school that are 30-45 minutes away. Since it is my right foot, I will be unable to drive.

My parents are amazing and will be helping out when they can. But they also have things going on in their own lives that will prevent them from helping at certain times.

We are looking into using some emergency nursing hours through our nursing agency to help fill in some gaps, but the nurses need to be on duty for a minimum of 4 hours per shift (realistically we would only need a couple hours at a time), so those extra nursing hours will be used up very quickly.

While this surgery is not an emergency, the orthopedic surgeon said that it should be done sooner rather than later.

I have a busy month at work (church) in October, starting a new curriculum for a program beginning October 20th, that I am now overseeing. I don't think we've mentioned on the blog that we made the difficult decision this past summer for me to resign from assistant teaching at the Ann Reid Early Childhood Center, so that I'm only working part-time at our church. Shortly after the school year started, I was asked to increase my responsibilities at our church from only leading Sunday School and Vacation Bible School, to overseeing all youth programs from preschool through 5th grade (which I accepted and am LOVING!).

So we decided that the surgery needs to happen soon, so that I am at least able to be up and on crutches leading up to the new program I am overseeing at church in October.

The surgery is scheduled for Tuesday, September 27th. Ten days from now.

Yikes.

Michael and I have our work cut out for us coming up with a schedule and routine to help the kids (mainly Ella) in the weeks following the surgery.

But I feel bad for Michael having to go through this process for the THIRD time in less than a year...working full time while taking care of the kids, house, dog, etc. while I'm basically sitting around doing nothing (for those of you who know me well, you know that I THRIVE on being busy and do not sit still very well).

So it will be a challenge. We're upset and frustrated that this has worked out the way it has with my foot.

But we'll get through it (we always do), and hopefully when all is said and done, my foot will be (just about) as good as new!!

Our Sasa (by Lindsay)

Many of you know this already from Facebook or word of mouth, but for those of you who haven't heard, we had to say goodbye to our beloved Sasa on August 24th.

We knew she had a heart condition.

We knew the end would be coming within 6 to 12 months (or possibly sooner).

It was sooner.

But luckily it was fast and she didn't suffer for very long.

She was fine until she stopped eating. A couple days later she couldn't keep water down and became extremely lethargic. The vet had told us back in June that these were the signs that would indicate that it might be "time."

We told the kids that afternoon that if she wasn't better in the morning, I would take her to the vet and we had to prepare ourselves that this might be the end for her.

Sasa didn't end up making it through that night. A little after midnight, she lost control of her bowels and had a hard time getting in a comfortable position due to her weakness.

And then she started crying.

Not a typical whimper like we've heard from her before, but a horrible gut-wrenching sound that told us she was extremely uncomfortable.

I called the emergency vet and we decided it was time to bring her in. We had hoped to wait until morning so that the kids could say goodbye, but knew that it wouldn't be fair to Sasa to make her wait several more hours until they awoke.

Luckily we had our night nurse on duty that night to stay with the sleeping kids so that both Michael and I could go to the vet (at 3:00 am). We decided to bring Potter with us so that our nurse wouldn't have to take care of him (he was wide awake with all of the action going on while we were trying to help Sasa).

As soon as we arrived, the vet immediately gave her a sedative to make her comfortable, but still be awake and alert. Then Michael, Potter and I had time alone with her to hug her, kiss her, tell her how much we loved her, and that she has been the most amazing dog we've ever known. It was one of the hardest things I've ever done.

Then the vet came in to give her two injections...the first was a powerful general anesthetic to put her in a very deep sleep so that she would not feel a thing. The second was a very high overdose of a sedative to stop her heart. It happened within a minute. Her loving heart stopped and we watched as she took her last breath.

We then had some more time alone with her before it was time to go.

The vet made an imprint of her paw for us to take with us as a keepsake. We are so grateful for that.

When we were leaving, we had to carry Potter out the doors, as he refused to leave. He didn't want to go home without his companion.

Potter went straight to Sasa's bed when we got home.

Later that week I made a shadow box containing everything "Sasa" that now hangs in our kitchen. It's a wonderful reminder of our first baby who we loved so dearly.

Then today (Sept. 17th) I had to bring Potter to the vet for an outbreak of hives (he's fine...just an allergic reaction that we can treat with Benadryl), but it was a very interesting appointment. Potter has been to the vet one other time after Sasa died to get his final round of puppy shots, and he was his normal, playful self.

But this time, we ended up in the same exam room where Sasa was put to sleep. While in the waiting room, Potter was his usual active and social self, trying to interact and play with all of the other dogs. But once we entered the "Sasa" exam room, his demeanor immediately changed. He lay down on the floor, softly whimpering. In the past, we have NEVER been able to get him to even sit still while waiting for the vet to come in.

After the exam, when we went back out to the waiting room with all of the other animals, Potter ignored them and lay down outside the "Sasa" exam room door. Dogs are incredibly smart. He remembered and misses his Sasa today.

Just like we all do.

Growing Pains...(by Michael)

As a teacher I've often heard and said that kids "grow up" so much over the summer.  And they do.

Summer provides a open forum for play, which as we all know, is the best way to learn.  It also allows for physical activity which strengthens the body.  Skills that require coordination can be practiced if not mastered over the summer.  Not to mention time for relaxing; those lazy summer days really do serve a purpose--to relax and recharge.

Kids also complain.  While there is a plethora of things they complain about there's one complaint that all kids end up engaging in...growing pains.  They often come at night, when the body is seemingly in rest but deep inside it's hard at work. Hard at work growing.  Hard at work taking all the days' activities and sorting through...finding value in one aspect and no value in another.  Muscles regenerate from the stress of play.  They strain and grow leaving their owner in a deep seated, dull, achy pain.  Kids cry from this throughout the night but it's sort of a rite of passage.

There's another type of growing pain that I never knew existed until now.  The SMA growing pains.  You see, kids with SMA fight many battles and they are not all physical. Many are internal battles they have with themselves.  These battles mature with age.  Ella turned six years old this June. Her mental battle took a leap and a bound over this summer. Her awareness of her disease, which lay relatively dormant up until this summer, began its ascent into her consciousness. And as the leg cramp creeps silently through the night only to settle squarely behind the knee, the awareness of SMA follows suit.  Only it doesn't settle in the leg but rather, in the mind.  As Ella grows she is able to perceive more of what is going on around her; she able to contemplate her own existence, she is able to question her lot in life~and she does.

SMA takes away what Mother Nature intended to grow.  Ella now sees this.  I watch her every day.  For moments she sits silent, brow furrowed ever so slightly as she watches her siblings play in ways she simply cannot.  She senses me watching and wheels over to me.  Usually she would avoid the subject altogether and distract herself with something else. But this summer she simply spoke to me about her feelings.  She's finished asking me why God allowed her to have SMA and has instead opened up with her feelings; her growing pains.

She's tired of her chair.  She's tired of her physical limitations. And she's tired of knowing that kids are meeting childhood milestones and as she approaches those milestones they become increasingly difficult to deal with.  SMA robs her. It robs her of typical development and she gets frustrated when she can't do something that she used to be able to do.

Now, as she gets older, she is starting to show me her anger. I want to say I understand but how can I? How can I say that I understand how it feels to have a disease that fights me everyday?  How can I say I understand having a disease that allows me one step forward and two steps back?  How can I understand the growing pains a kid with SMA must be going through?  I cannot.

We talk. She grunts. We reason. She hits her legs with her hands. We hold hands. She "charges". 

At night, when the other kids are crying about their growing pains, Ella cries for hers...yet her knees aren't what's bothering her...her uncertainty is.  She doesn't want to be alone at night; she doesn't want to be left lying in her bed, unable to really move herself while experiencing all the typical night-fears all kids have.  But for her, she cannot "run" away from her night-fears into mommy & daddy's room, or even Ava or Henry's; and now she knows it all to well.  Often, when I say goodnight to her, her mouth frowns as she fights not to cry. I can feel her growing pains building and the fear dwelling in the room.  I kiss her and part; leaving the door open and the light on low.  

Her nurse still comes and is absolutely wonderful.  She, too, noticed the "growing pains" of Ella.  Just recently, however, she has made some suggestions for us to try to help Ella fall asleep. It has been working just great.  And with good sleep comes good days.  She is not as angry about SMA; she is not so "tired" of her chair, her limitations, and her challenges.  In fact, she has decided to face her challenges head on.

The video below shows what a determined, intelligent, and inspirational child Ella really is.  She worked this problem out all by herself.  Relieving Mommy, Daddy, Ava, and Henry of having to open the door for her to go in and out of the house to and from the deck.  

We live with Ella and she simply blows our minds away all the time...this one's really great!

A Pathway...by Michael & Lindsay

We all know that life "has its bumps".  For Ella, that is a reality she lives with every single day.  We are so fortunate that so many people and several organizations have stepped forward to help our family.  We honestly wouldn't be where we are had it not been for the care, dedication, and pure kindness that has surrounded us. 

Ella is six years old.  She LOVES to play with her brother and sister, with her friends, and with her neighbors.  Of course, kids being kids, they run and jump and play all over the yard and the neighborhood. Ella does everything she can to keep up with them...and does a great job of it!

We have noticed that as she cruises around the front and back yard areas she is pretty good at changing her pathways, thereby not wearing muddy paths in either of those places. While this has always been the case, it is not so for the pathway she has forged allowing her to go back and forth between the front and back yards; the lawn in this area (which is on the side of the house) really only allows for one path for her because of its width.  This small area is being worn down and the grass is dying, leaving sloppy muddy spots where her chair can get stuck.  Of course, rainfall coupled with very poor drainage in that area only exacerbates the problem--and it promises to get worse.

Several organizations have tried to help but to no avail thus far.  So we have decided to tackle the problem on our own.

What we aim to do is create a structured path from the driveway to the playground in the back yard using rubber mulch. Rubber mulch lasts longer than wood mulch as it is guaranteed for 12 years.  It's cleaner and provides a smoother, more cushioned-based path for her to travel over. A stated before: without a structured pathway, the grassy area Ella uses to go from front to back (and vice-versa) will soon become a sloshy, muddy mess; eventually she won't be able to use that path since her power wheelchair would get stuck in the mud. 

Taking this all into consideration we have decided to reach out to whomever might be willing to help.  In general, we are looking for anybody who might give us a few tips on building a rubber mulch pathway (~45' long x 3' wide x 2" deep). We are also going to install a little extra drainage to that area (catch basins, some short underground piping, & a pop-up drain) to redirect the puddling water. We have done a lot of research.  We even talked with a man, by chance, who designed the Morton Arboretum's children's play area (he now works at Home Depot).  We are ready to move forward.  If there is anyone else out there who has experience in this type of work and can give us advice, tips, or tricks--it would be greatly appreciated.

Please feel free to email us at:  mcc2oreos@gmail.com if you or somebody you know, would have anything to share that would be useful to us as we embark on this project for Ella.

Here are 2 videos; the first is showing the bumpy path she's forged on the side of the house.  The second shows how nice of a ride she gets on the rubber mulch in the playground area. There is also a picture showing the area we plan on draining & renovating for her.

Videos and Pic