Chasing Dreams...(by Michael)

Ella will be attending MDA Camp for the second summer in a row this summer! Last year she was hesitant to go because she didn't want to leave mommy and daddy for a whole week. She had a couple rough nights in the beginning but soon got over her fear. This year...she's ready!

Just recently, we got an email from one of the lead counselors of the MDA Camp asking us to share Ella's dreams. This is something they do every year to get to know the kids as well as encourage their dreams.

Ella has many dreams for herself. She wants to be a singer, she wants to walk, she wants to be independent, she wants to get married and have kids, and more. I had a whole list of ideas to share with the counselor but had to chose only one of them.

I decided to go with Ella's dream of getting married and having kids. Ella loves weddings; as we went to one over this weekend. She was so enamored with the bride, her dress, and what it meant to be a bride. 

Right now, however, Ella is all about her friends. She wakes up in the morning and tells me she has to be at a certain friend's house at a certain time. She does this so she doesn't have to worry about ringing the doorbell which might be out of her reach...if her friend is expecting her at a certain time then they'll be watching out for her. So far, she's been right on time--every time.

She often spends the day away from home. She has a Gizmo which is a little watch-like device that acts as a phone. She calls me every time she is going to do something new or go visit another friend. When she needs to use the bathroom she races home in her power wheelchair and let's me know she needs to use it...and I take her. During that bathroom time she tells me everything she and her friend(s) are doing. She is really polishing her social skills.

Yes, one of Ella's dreams is to get married and have children. The social skills that she is developing are all important to that end. She has to find ways for people to look past her disability and see her for who she is as a person. She needs to learn how to advocate for herself as she won't always have mommy and daddy to help. She needs to learn how to take disappointment well, so that it doesn't slide into her disability...where she could use her disability for blame. As she grows, so do her social skills.

Ella loves children. She is willing to play with any aged kids in the neighborhood. Each day she finds herself with different aged playmates. And she adjusts herself to that age. When with the younger kids she becomes their mother, taking care of them and teaching them things. When she's with same-aged friends, she adjusts herself to be present just as she is. When playing with older kids, she once more adjusts herself to communicate with them at their level. And when she's with adults she turns on her humor and polishes her sarcasm!

Ella may or may not get married and have children. But whatever she does she will continue to chase her dreams, even if that means chasing them down in a 400lb power wheelchair. 

Ella at a friend's wedding (June, 2019)

Privacy and Dignity...(by Michael)

Taking care of Ella requires a lot of lifting, transferring, and adjusting on our part. She's is like everybody else and needs to move in order to be comfortable. This movement occurs all throughout the day and several times at night. We know that there are lift systems available and maybe one day we'll get them; but for now we simply lift and move her.

Over the course of the day, like you and me, Ella needs to go to the bathroom. She drives her wheelchair to the bathroom door on the first floor and we lift her out of her chair and onto the toilet. There's two ways to get her ready for the toilet. 

• The first requires holding her by the waist and lifting her up against our body while she wraps her arms around our neck. We then pull her pants down and place her on the toilet seat. This method makes us arch our back a bit in order for her to get a good grip around our neck and for us to balance her as we pull down her pants. Getting her off the toilet seat is done the same way...we lift her from the toilet by the waist, arch our back a bit for her to grip around our neck and to maintain balance, then pull her pants up and place her in her wheelchair.  

•  Another way to do it is to scoop her (getting one arm behind her knees and the other arm behind her neck to lift) out of her wheelchair and lie her down on the landing of the staircase. Bending over her, we take down her pants and then scoop her from the landing and bring her across the hall into the bathroom to sit her down on the toilet. This requires a lot of bending and twisting on our part. With this method, if there are people in the house, she wants them to close their eyes and/or turn away so they don't see her half naked body as we go from the landing to the bathroom.

Lindsay already has a bad back with a degenerating disc so lifting Ella can aggravate that area. We do our best to use proper lifting techniques but that doesn't always happen. If our backs go out then we have a problem.

Fortunately, we had a solution (something we thought about doing when we first bought this house) to getting her in and out of the bathroom on the first floor safely--while maintaining her privacy and dignity. Our idea was to expand the bathroom into the adjacent hall closet. We could never afford to do this on our own so we decided to use the recent donation money for the job! Construction workers opened the shared wall between the bathroom and hall closet and expanded the bathroom size by approximately 15 square feet. In that expanded area we placed a modest dresser with a soft cover on top. So now, we simply (and correctly) scoop Ella from her wheelchair and place her gently on the dresser (which is waist high on us). We get her ready and then easily transfer her from the dresser to the toilet using only our legs to bring her down to it. We do the reverse when she's finished...scoop her from the toilet to the dresser, pull up the pants, and transfer her to her wheelchair. The workers also created a new hall closet on the opposite side of the new wall  (see pics).

We would like to thank all the people who have donated  money to us so that we were able to pay for this major bathroom modification that we've needed for years. Not only does this help us physically, it helps Ella emotionally as well--as she now has the privacy she needs and the dignity she deserves while getting ready for the toilet. 

Before (bathroom/closet shared wall is just to the left of the toilet)

Taking out the shared wall between bathroom and hall closet

New Hall Closet

New bathroom with private changing station (safety, privacy and dignity intact!)

Compassion and Generosity...(by Michael)

For kids, the world can be a scary place sometimes. When they witness bad things happening to others they often wonder why it has to be that way. 

Our own kids (Ava, Henry, and Ella) have had to watch their mother fight a battle against a rare and aggressive infection for four years. Then they had to be witnesses to her leg being amputated and all that comes with that. In addition to those events, they have to constantly battle against SMA each day.

On the other hand, they also get to witness the good hearts of people, in our Naperville Community (Church & Prairie Communities) and other communities, many people of whom they don't even know, entering our lives and providing a helping hand. They also witnessed family rallying around us. This experience teaches them compassion and generosity.

I work as a teacher in the community of Elmhurst, IL. I have been at the same school and the same grade (4th grade) for 19 years now. And when Ella was first diagnosed with SMA, the community rallied around us and threw a fundraiser that allowed us to buy a modified van for Ella. We are forever grateful to those who participated. 

When Lindsay lost her foot to the infection--and the community of Elmhurst learned about it--they once again rallied behind us and provided support. Parents from my class of 2018-19 put together care packages for our kids. So much support came flooding in from the Elmhurst Community that they had to present us with care packages over a period of time; every few weeks. These care packages included games, coloring books and coloring supplies, toys, snacks and candy, s'mores ingredients, and a new video game system. We were taken aback at the generosity of those involved in supporting us. 

Our family, church, and Prairie Communities also took hold of the opportunity to help us and offered meals, financial assistance, good thoughts and prayers, and neighborly help.

Ava, Henry and Ella have a lot on their minds these days. They wonder about the future of Ella and what that means for each of them. They inquire about what their mother will have to go through with two more surgeries coming up. They look forward to when Lindsay gets her prosthesis so she can walk without assistance. 

And even though some bad things have happened over the past four years, it's comforting for all of us to know that there are so many people out there ready and willing to offer support; whether it be via care packages, meals, lemonade stands, financial support, helping with medical appointment transportation, babysitting, or prayers and positive thoughts. 

Every bit of it helps. 

 Friends from Elmhurst Delivering Care Packages

Ella's Birthday Gift...(by Michael)

Lindsay and I sat in the doctor's examination room with our baby one-year old Ella by our side. The doctor was looking at some test results and was deeply engrossed in what he was reading. Finally he said that Ella most likely has Spinal Muscular Atrophy and that there's nothing they could do for her. He advised us to take her home and love her until the disease takes her away from us. We left the hospital devastated. The actual, official diagnosis of SMA came after genetic testing was completed...that day will live in our minds forever.

Ella became sick shortly after that visit with RSV (an upper respiratory virus) and ended up being hospitalized at Children's Memorial Hospital of Chicago (now Lurie's Children's Hospital). While we were at the hospital we heard that a prominent neurologist was going to be working there. We made a follow-up appointment with her. She showed us how to take a proactive approach to the disease and set us up with several machines and resources.

Eight years have passed and Ella is still with us. She will be turning 9 years old tomorrow (Monday, June 10th). Over the past eight years Ella has been through much. She has been hospitalized and undergone three major surgeries. She has worked really hard in her therapy sessions week after week. She's learned to maneuver a 400 lb power wheelchair and drives it better than most people drive their cars. She's thrives at school and continues to make friends. She cruises the neighborhood and always has a smile on her face.

When it comes to being independent Ella has made wonderful strides. She recognizes what she can and cannot do for herself; and when trying something new she always puts forth great efforts. She's smart, likable, and fun to be with.

Within the past few weeks Ella auditioned for and made the Young Naperville Singers that will start in the Fall. She is excited to have an extracurricular activity like Ava and Henry do, for herself. Since we know she'll be doing a lot of singing we decided to get her a karaoke machine for her birthday. We didn't have a lot to do over the weekend so we decided to let her open her birthday gifts a few days early. She tore right into them. She received books, a stuffed unicorn, several boxes of kids stick on nails, and the karaoke machine. Needless to say, the kitchen table is full of the gifts as the girls put the nails on and all of us sing into the karaoke machine. During more quiet times, she reads her new books. And, of course, she keeps her stuffed unicorn with her day and night.

On Ella's actual birthday she has to go to the doctor to get the rods in her back lengthened...a relatively easy procedure that is outpatient. They simply use a magnet on the outside of her back to lengthen the rods on the inside...she's really growing up! From there we'll go out for a family dinner at a place of Ella's choice.

SMA has been a tough road for us to handle. Many times we were less than confident in our ability to care for Ella. We turned to family and friends and began building a network of support that stands strong to this day. We learned all we could about SMA--from what causes it to the plan to cure it. We've been the fortunate recipients of fundraisers, lemonade stands, donations, and support for our whole family.

We started this blog to let people know what's happening, both far and near. To this day we have 478,865 hits! Thank you for taking the time to read our story...please feel free to share this blog as you see fit as a gift to Ella!

Web address to copy & paste & share: 

https://ellas-corner.blogspot.com/2019/06/ellas-birthday-giftby-michael.html 

Ava, Henry, and Ella...(by Michael)

Lindsay and I have three children...Ava (11), Henry (10) and Ella (8). They live a life that is quite different from their friends. Having a sibling with SMA (Spinal Muscular Atrophy) has weighed heavily on Ava and Henry's minds and their behaviors. We have talked with all three of them about the implications of Ella having a terminal illness and while they understand those implications they still have to live with the disease everyday. Often there is jealousy as Ella receives more attention than they do. There is resentment for having to help her with everyday tasks. There is anger and hostility surrounding the harsh facts of the disease. It's a lot for them to handle just in itself.

All three children have also had to deal with their mother going through significant health issues for the past four years. With each surgery we told them that we hoped it would be the last. This has proven to be untrue. They have gotten used to the foot surgeries, the recoveries, and the aftermath. But kids often keep their emotions to themselves and worry quietly.

When Lindsay had her lower right leg amputated the kids were scared; afraid of the unknown. They had no frame of reference to process the idea of an amputated leg. When all was said and done they rose to the occasion and supported their mother. They helped with things and took the light side of it, following Lindsay's lead. When others offered help through care packages, meals, and GoFundMe donations they were so grateful; their joy was infectious.

On June 4, 2019 Lindsay was diagnosed with an 80% chance of having Stage 1 Renal Cell Carcinoma (cancer). The doctor is going to remove the tumor and 5% of her kidney on July 8, 2019. 

The kids are scared. Once again, they really have no frame of reference to draw upon to process this news. They know that cancer is bad and they feel unsure. We spoke with each of them about how they feel regarding the two upcoming surgeries (the first one is fixing Lindsay's torn meniscus in her left knee and the other one is the removal of the tumor). 

I sat with Ava in the family room chair that can fit a child and adult together. I asked her how she felt about the upcoming surgeries and situations. She told me that she was fine with the knee surgery. I asked her how she felt about the cancer issue. Then she paused, put down her iPad, and scooted closer to me. She let out a sigh and wrapped her arm around me in the front. I squeezed her closer and gave her a kiss on the head. No words were spoken but I could feel her anxiety in the hug. I told her that it'll be fine. She shook her head and tightened her grip on me.  We sat in silence for a moment and then she picked up her iPad and continued playing her game. She's a quiet girl at times and right now she's burying herself in her technology. 

Lindsay had a conversation with Henry. She asked him how he felt about all of this and he replied that he was ok with the knee surgery but was "ish" about the cancer. Henry is a very affectionate boy and often wraps himself up in Lindsay's lap. He, too, tends to hold things in until they explode--usually in anger. He takes a lot of his frustrations out on Ella and blames much on SMA. He ends up storming away...retreating to his room to cool off. 

I asked Ella how she felt about Lindsay's situation. She, too, was fine with the knee surgery but hesitated when asked about the cancer. She took the point of view that they are going to get rid of it once and for all. She lay on the landing as we talked, her big brown eyes sinking deeply into mine. she said, "I think it'll be alright." Ella is the type of person who likes to talk about things. She gets this from the many conversations we've had regarding her own health. She's mature in that way beyond her years. When frustrated, she tends to bother Henry and Ava and then gets herself all worked up and cries. Sometimes I feel that this crying is more than just a reaction to a sibling rivalry, but more an expression of her emotions overall.

Each of our kids respond to stressors differently. Ava sinks into her iPad, Henry tries to spend time with friends, and Ella works on creating, games, and make-believe; she also is trying to expand her friendships. They all, however, tend to hold emotions in until they can't handle it anymore and they seek an outlet...anger, crying, indifference. We've made it a point to try to have family time together playing games or eating meals with one another. These times, though, are getting farther and farther apart as we also have to continue to run a household. Lindsay does as much as she can and is still adjusting to life with an amputated leg. 

As parents we are dealing with delicate psyches...all in critical developmental stages. We interject humor and seriousness, discipline and leniency, raw facts and hypotheticals. Each child of ours comes to us with their own view of the world...and through the thick of what's going on we try to make what they see coincide with how they feel. Often the two are quite different.

Henry (10), Ava (11), and Ella (8)    Summer, 2019

Overflow...(by Michael)

Yesterday (6-3-19) I waited at home while Lindsay went to see her orthopedic surgeon to have the 28 staples removed from her amputation incision. I was hoping all went well and waited anxiously. She returned and the first thing out of her mouth was that she needed surgery on her left knee. The meniscus in her knee had torn once again from compensating due to pain. We knew this was inevitable but still cringed at the prospect of yet another surgery. Her staples did come out without any incidence. The knee surgery has been scheduled for this Friday, June 7th. It should be a routine procedure with a comparatively easy recovery. Performing the surgery now will allow for her left knee to be fully healed and strong for when the time comes to get her prosthetic leg. 

We still had an appointment today, on June 4th, with Lindsay's urologist regarding the mass found accidentally on her kidney. This mass was discovered after she had a bad reaction to the hyperbaric oxygen pressure chamber treatments and they did a CT scan of her torso (followed by an MRI to further investigate the kidney mass). Finding this mass was a blessing in disguise, if you will.

Lindsay and I waited in the examination room of her urologist ready to take in whatever news he had. He entered and gave his greetings and we got down to business. The doctor pulled up the MRI scan that was taken two months ago and showed us the mass that was sitting right on the top of her kidney. He spoke a little bit about it in terms of its size and location. He then gave us three choices:

• We could wait and watch it to see what it does.
• We could have a biopsy done to see if it is malignant or benign.
• We could have the mass removed in its entirety.

Lindsay grabbed my hand as we went through each option. Waiting to see what it does didn't really appeal to any of us. Since the scan was two months old we don't really know what it has done since and waiting would cause unnecessary worry. We considered a biopsy for a moment until the doctor told us that even a biopsy could be diagnostically inconclusive. And furthermore, whether it is malignant or benign, it would still need to be removed. The last option seemed like the most logical choice. The doctor recommended to remove the mass in its entirety.

Upon further conversation the doctor let us know that he felt it had a 75-80% chance of being Stage 1 Renal Cell Carcinoma (cancer) based on its location and presentation. Lindsay squeezed my hand tighter and tears started welling up in her eyes, as well as in mine. I squeezed her hand, rubbed her back, and offered her a reassuring look. The doctor told us that it would be a relatively easy arthroscopic procedure. 

We left the doctor's office with our fears confirmed. Earlier, we entertained the thought of a cancerous tumor but didn't really want to believe it. We opened the papers that explained Renal Cell Carcinoma and stared at the drawings of her case on the printed picture of a healthy kidney. The doctor's plan is to remove the tumor and about 5% of her kidney at the site.

Another surgery to go through. Another recovery to endure; about 4 weeks. 

After the surgery, assuming the pathology reports are negative around the border of the mass, Lindsay will have annual MRI scans to monitor surrounding systems for any signs of malignancies. 

The kidney surgery has been scheduled for Monday, July 8th, while the kids are at camp for the week. Lindsay will have to stay overnight at the hospital for a night or two. 

Our plates are full and yet another serving has been added---our plates overflow.

To help offset medical expenses please follow this link to our GoFundMe Page

She's Amazing...(by MIchael)

Before Lindsay had her lower right leg amputated (due to a four year long foot infection) we did a lot of research about amputation procedures and aftermath expectations. One of the things that we found was that there is usually a period of time where the amputee grieves for the loss of the limb. But this grieving was more prevalent in those amputees who lost a limb unexpectedly. Lindsay had an advantage as she had time to process the idea of an amputation.

It's been 23 days since her amputation (as of 6-2-19) and her recovery is going smoothly. She is extremely mobile using her wheelchair and her walker. And she is doing things that we never thought she'd do in such a short time frame. 

She's amazing...with three kids under the age of twelve dwelling in our house and the end of the school year upon us the house was quite chaotic and messy with school supplies and school work from the year strewn all over the place. Our kitchen table was full of "stuff" and I was overwhelmed as to where to put it all. I started with a few things and found a place for them but there was still a pile looming there and elsewhere on the first floor. When I woke up this morning and went downstairs, the kitchen table was completely cleaned off and there were fresh flowers as the centerpiece. Lindsay had also decluttered the entire first floor on her own the night before.

She's amazing...during the last few weeks Lindsay has been sleeping on the living room couch so she could be present in the morning to send the kids off to school without having to go down the stairs. Once summer started she moved back upstairs to our bedroom. That last day of school she took the bedding from the couch and washed it all...by herself! 

She's amazing...she has employed a contractor to install extra stair railings so she can go up and down the stairs in the house (both the upstairs stairs and the basement stairs). She can now be free of a crutch when she travels the different levels of the house...and it's safer for her as well. 

She's amazing...in order for her to care for Ella when the time comes she knew that there needed to be some modifications completed in our first floor bathroom. She quickly planned these modifications, contacted a contractor, and put into motion the necessary timeline for the modifications to occur. We will be expanding the bathroom into the adjacent closet in order to put a changing table in there for Ella. Lindsay has a degenerating disc in her back and bending down on the stair landing to get Ella ready for the toilet would wreak havoc on her back (not to mention mine). 

She's amazing...from her computer that now resides in the dining room and a floor workspace she made in the same room, she is getting ready for VBS (Vacation Bible School). She is making props, coordinating staff members, and compiling registrees. She is running her job at church from home, making sure everything is working and running in the way she wants them to.

She's amazing...not only is our house filled with three kids and their friends, we also have two young dogs and a bunny. The cage for the bunny is in the corner of the living room. I walked in from school one afternoon and there was Lindsay, on the floor, cleaning out the bunny cage and getting everything nice and neat. 

She's amazing...taking a shower with half of your leg missing can definitely present a challenge and also be quite dangerous. Lindsay set up our master bathroom with organization shelving and a bath chair that allows her to be independent in taking a shower and getting herself ready for the day. She has everything at her fingertips and works the wheelchair in the bathroom like a pro.

She's amazing...amputation of a limb can be a horrific endeavor. Lindsay decided to take the light route instead and created t-shirts (using iron ons) with sayings that poke some fun at amputees ("I always leave my leg in my other pants", "3 out of 4 isn't bad", "I'm stumped", etc.) She has the attitude that if you can't laugh at yourself then who can you laugh at?

She's amazing...I have a rock in my classroom that says, "Attitude is Everything" that was given to me by a former student. I use that saying often with my class. Lindsay embraces that saying as she virtually skipped the grieving stage of the amputation...oh sure, there were and are times when the concept of not having a foot weighs heavily on her mind, but she talks it out with herself and sometimes with me in order to get herself back on track.

She's amazing...I watched my wife battle a nasty foot infection for four years. The pain, discomfort, emotional and physical toll were often too much to handle on our own. We turned to others for help and the relationships that Lindsay and I forged over the years became evident as people stepped forward during that time to offer help. And more so, when the amputation took place, other people came forward...giving us the help we needed to traverse this part of our life. 

She's amazing...for the past four years the infection of her right foot was all-consuming in our life. It dragged her down, took its anger out on her physically, and made life a terrible mess. Instead of looking at the amputation as a loss of a limb she looked at it as a loss of the infection. She thrived physically and emotionally after the amputation, grabbing hold of her life again.

You're amazing...all of our readers, those subscribed to our blog, those who keep in touch through Facebook, those who happen to come across our blog in a search, those who are part of my teaching community (Elmhurst), and those in our Prairie community...all of you are amazing in that you have committed to thinking about us, praying for us, and supporting us in so many ways. She's amazing because you're amazing.

This time in our lives is definitely a challenge...emotionally, physically and financially. So many people have come forward to help with our CareCalender and our GoFundMe Campaign. So many have come forward to support our kids with care packages full of toys, books, and snacks. So many have provided meals. So many have come forth to offer their thoughts and prayers. So many have come forward to help us financially as medical and equipment bills pile up.

It's honestly a testament to how amazing Lindsay really is... 

Summer's Upon Us...(by Michael)

The smell of freshly cut grass. The warm winds blowing through the trees. Birds chirping and squirrels scampering. People taking leisurely walks and the sound of the ice cream truck in the neighborhood. Spring is here.

For months our kids have been inside playing as the winter took over. For months they have been on their devices, or playing board games, or watching movies and crafting. The time has come for them to venture outside.  

Over the years the kids have built a network of friends from school. They text one another through the messaging apps or converse through games...making plans to get together. In one moment the kids will all be home and the next moment they’re on their way out the door; off to a friend’s house.

Even though Ella is in a wheelchair, this doesn’t stop her from making friendships and playing outdoors. The trickiest part for her is initializing the get-together when she wants to play with someone. She cannot simply drive herself over to a friend’s house to see if they’re home--she can’t get close enough to reach the doorbell. She often relies on us to text her friends’ parents to see if they’re available, or even has us walk her to the friend’s house. Sometimes she’ll take a chance and simply roll herself out the door, down the driveway and go straight to a friend’s house to see if they’re outside playing. She brings her Gizmo (a watch/phone) and calls us when she knows if she’s staying. She is ever hopeful. Once she's there the families make great efforts to accommodate Ella and her needs.

Ella has another challenge that faces her when it comes to playing with her friends. The bathroom. Often, after being gone for an hour or two, she has to go to the bathroom. She’ll call us on her Gizmo and ask if she and her friend can come to our house for a while. She often gives the friend a ride to our house on the back of her chair. They roar down the sidewalk, Ella navigating every bump as she has it all mapped out in her head. She rolls up the driveway and lets her friend off. In the house they go straight for the pantry to get a snack. Once her friend is taken care of Ella will ask me to take her to the bathroom. I oblige. When that’s finished, the kids might spend a few more minutes at our house (depending on what they find to do) or simply race out of the house to continue their day of escapades.

Ella has a whole summer ahead of her. She loves to be outside racing around in her power chair, or tooling around in her little chair. She enjoys the sprinkler, groups of friends playing together, and having cookouts on the deck. One of her favorite past times is swinging on the swing. She has learned how to sit and balance on the swing (ever since her back surgery) and thrills at the prospect of swinging back and forth as I push her, whooshing through the air. Kids need that kind of movement to help build their vestibular systems (that’s why they love spinning and being upside down); kids with SMA often miss out on those kinds of movements.

Ava will be in 7th grade, Henry in 5th, and Ella in 4th next Fall. These are all ages in which friendships and socializing are important developmentally. We are fortunate to live in a neighborhood where there are plenty of kids and families around us...we’re especially lucky that these families are willing to adjust themselves to Ella’s needs.

It's All About the SMN...(by Michael)

“Our job is not to save her, but to keep her alive until they find a cure.”

Those words were spoken between me and Lindsay on the night we found out Ella had SMA. We were in a place that no parent wants to be...knowing that your innocent child has a terminal illness with no cure or treatment.

If you go back onto our blog pages and look at the historical entries you can see the journey we have taken is one of harsh realities around many corners. We watched as our daughter, our “Squishy”, lost more and more abilities. We watched as she struggled through her days and her nights. We watched as the delicate fabric of life came undone before our eyes.

As time passed we got Ella on an Amino Acid Diet, got her into therapies, and took a proactive approach to her care; only then did we see a plateau effect in her skills and abilities. She didn’t seem to be losing skills nor gaining them either. And things always got worse when she had her surgeries. The surgery recovery came at a cost---namely the loss of skills and increased contractures.  

Now we have Ella on the only treatment for SMA known as the drug, Spinraza. We are seeing improvements in her strength and skills and are very grateful to be able to offer this treatment for her. She has to have injections of Spinraza every 4 months for it to be effective.

For eight years, the physical toll SMA has had on me and Lindsay cannot be put into words. Every day it feels like Ella gets heavier and heavier. Lifting her, transferring her, and re-positioning her takes its toll on our bodies.

SMA has more than just a physical impact on our lives. As a family, we have struggles with Ava and Henry and their reactions to having a younger sister with SMA. They often feel left out because Ella gets so much attention from so many people. They feel resentment for having to have to help Ella with the simplest of tasks. Their ability to empathize with Ella is minuscule at best. They harbor anger. They harbor jealousy. They harbor little sympathy. It’s a lot for them to handle.

Lindsay and I have our battles to fight when it comes to living with SMA as well. We both have to reconcile within ourselves the acceptance of the disease and its implications. We have to balance the care we provide for Ella and the care we provide to Ava and Henry. We have to also take care of our relationship with each other. Having a child with a terminal illness who is so dependent on you for so many needs takes so much time, energy, and emotion; often there is little left of any of them for maintaining a healthy personal relationship with your spouse. Every minute counts, no matter how few and far between they may be.

SMA is caused by a missing or mutated gene. This gene (SMN1) is responsible for producing a protein known as SMN (Survival of Motor Neuron). This protein keeps our motor neurons alive. A person with SMA is either missing the gene or it has mutated. Over time, since there is no SMN protein being produced (or very little is produced by back-up gene[s]), the motor neurons of those afflicted slowly die off, or go dormant (doctors aren't sure if they die or just go dormant).

Today (5-24-19) it was announced that the first ever gene replacement therapy has been approved for children (infancy to two years old)  with SMA. This drug (Zolgensma) would replace the missing or mutated gene, thereby producing the needed SMN (Survival of Motor Neuron) protein thereby keeping motor neurons alive! The drug would not reverse any damage but it will "change the course of SMA" [Kenneth Hobby--CureSMA].

We’ve waited eight years for this breakthrough and it is finally upon us. Waiting some more time for the FDA to approve it for older children and adults will be worth the wait if it saves lives. In the meantime, we do the best we can to take care of Ella.

Click here to learn more about Zolgensma.

Nine-One-One...by Michael

The day was a cold and dreary Sunday. Clouds hovered overhead all day long threatening rain. The wind blew little white flowers off the tree in our neighbor's back yard. Lindsay and I decided to go out on our back stoop to sit and talk, like we've done a million times before. The stoop leads to a pea gravel area surrounded by a fence (to keep the dogs in). 

Lindsay has a pair of forearm crutches and she decided to use them because they left her arms free to do things. We sat with each other and talked about our life, what's coming next, and how we're going to handle everything. A few sprinkles found their way to us and we decided to go inside before it started really raining. Lindsay started to get up on her one foot and grabbed the crutches and put them on. She lifted herself onto the stoop and reached one hand toward the house for support. One of the crutches slipped on the wet pavement and her body twisted in an attempt to right herself, the crutches, still attached to her forearms became tangled in her body. Her amputated leg was right over the stoop. Then she fell toward me and her stump landed square on the stoop taking all of her weight. She let out a scream and her body began collapsing. I tried to grab under her amputated leg to protect it and ended up cradling her in my arms. I let her head down gently onto the pea gravel and lifted her legs up. She was on her back screaming and crying, her eyes rolled back in her head a bit and she had a hard time catching her breath between the screams and cries. She grabbed for her amputated leg and let out a wail while asking, "Is it bleeding, is it bleeding?". We started to unwrap the Ace wraps that were wrapped around her stump looking for signs of fresh blood. My heart started racing as she continued to cry and scream, "It hurts, it hurts!!". 

Just then the rain started coming down without any warning. I could see droplets falling on her shirt and felt the drops falling on my back. Between the two of us we got the Ace wraps off and got down to the dressing. She pulled her stump toward herself and looked at the end of the stump. There was only dried blood from the days before. No fresh blood. She screamed again and more tears flooded out of her eyes and down the sides of her head. The rain kept pelting us with big thunderstorm type drops. I wanted to see if she could get up but had no idea on how to help her. I felt helpless. She started rolling from side to side holding her amputated leg. I took out my phone and dialed 9-1-1.

The operator asked me where the emergency was taking place and I gave her our address. She asked what the nature of the emergency was and I told her that my wife just had her leg amputated a week ago and she fell right on it. The operator assured me that the ambulance and fire truck were already on their way. She kept me on the phone, asking me questions about Lindsay's consciousness and if there was any other injury from the fall. I answered her questions holding the phone in one hand and placing my other hand on Lindsay's thigh so she knew I was still there. 

Within 5 minutes, which felt like an eternity, I heard the sirens down the street. The noise grew louder and the rain grew stronger. Our shirts were becoming soaked and Lindsay continued to writhe in pain. The paramedics arrived and went into the front of the house and the kids, who had no idea what had happened, saw the first responders and were taken by surprise. I heard the dogs barking their "warning" barks and went into the house to get them away from the paramedics. The operator had told the paramedics that we were on the west side of the house. I made my way back to Lindsay and as I got to her a couple of paramedics were arriving with a stretcher. The stretcher was having a hard time in the wet, muddy grass so they stopped it on the other side of the fence and went through the little fence doorway to Lindsay. They knelt down beside her and began to assess her injury, asking if she hit her head, asking her her name, asking her the day. She answered all of the questions correctly. The paramedics decided the stretcher wouldn't fit though the fence doorway and produced a small red tarp with handles on the ends. They rolled Lindsay to the right and placed the tarp under her back then rolled back to the left and onto the tarp. Two paramedics grabbed the handles and lifted Lindsay off the ground. They brought her through the fence doorway and placed her gently on the stretcher. They decided that they would have a man at each corner of the stretcher in order to move it safely through the rain soaked grass. Two firemen and two paramedics took their places and got the stretcher moving.

They rolled the stretcher to the ambulance and I followed behind. We were having a church friend come over to help with some vacuuming and she had just arrived. She stood on the sidewalk and watched as they rolled Lindsay by her, a look of disbelief in our friend's eyes. By the time they got Lindsay into the back of the ambulance the kids had come out onto the driveway. Our friend cradled Ava in her arms giving her a side hug. Ava's hands were over her mouth. Ella was there in her power wheelchair trying to get a look at her mommy. I put my hand on Ella's head and told her that mommy would be all right and they just had to check her for injuries. Henry was at a birthday party and had been driven by another church friend.

I was called into the ambulance to speak with Lindsay after a few minutes of the paramedics' initial assessment. It was decided that she should go to the ER just to make sure that no damage had occurred. Lindsay agreed and I told her I would follow her to the hospital. I asked our friend if she could stay while we went and she said that would be no problem, "Go!" she said, "Go!".

I arrived at the hospital and had to wait while they processed Lindsay into a room. I was called after about 10 minutes of waiting and given directions to her room. When I arrived she was lying on the bed with her stump exposed. This would be the first time either of us has seen the stump without dressings on it. The end of it was smooth and the incision was wrapped around the front of it, held together by 28 staples. Everything was intact; no blood anywhere. 

The hospital took an x-ray to make sure everything inside was good and it was. Lindsay was given 2 doses of very powerful pain meds and we waited for the bureaucracy of an ER to make its course. We were there for 2+ hours. They redressed the stump and sent us on our way.

Lindsay was in significant pain throughout the night and into the next day. She had an appointment with her orthopedic surgeon the next day and he couldn't believe that nothing happened to the stump given the sheer force that was thrust upon it.

Our lives are up and down these days and instead of taking everything one day at a time we are now taking everything one hour at a time. We're so grateful for the Naperville Fire Department for taking such good care of Lindsay as well as the ER doctor and nurses. We are especially grateful to our friends, who are two of many, that they were there to help take care of our kids.