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. 

Life Goes On...by Michael

We have six wheelchairs in our house, two walkers, three pairs of crutches, a cane, a knee scooter, and an iWalk2.0! Four wheelchairs belong to Ella, one of the pair of crutches belongs to Henry and the rest are Lindsay's.

Lindsay came home from the Marionjoy Rehabilitation Hospital today (5-18-19)...and brought all of her necessities with her. When I arrived at the Rehab Hospital Lindsay was sitting in her wheelchair finishing up her last bit of packing. When our eyes met we both broke into smiles and I went forward to give her a hug. I started to straddle the footplates of the wheelchair when Lindsay said, "Uh uh, I'm standing up for this." She placed her arms on the arms of the wheelchair and got herself up on her one leg. She wrapped her arms around me and we hugged like we've hugged many times before.

Just then a nurse entered the room and found herself a seat. I sat down as well and we proceeded to go over the discharge instructions with her. She talked about safety, complications, and whom to call when questions arise. At the end of it all Lindsay had questions for her and they were answered. Lindsay signed the paperwork and she was free to go. Another nurse came in with a cart and we piled Lindsay's belongings on it. She said a few more goodbyes to the staff that was there and we were on our way.

On the way home we stopped at Oswald's Pharmacy because they have medical equipment of all sorts there. We picked up Lindsay's second walker and bought a second wheelchair (both for upstairs). We piled everything into the car and went home where the kids were waiting anxiously for their mother. 

Over the past few days Lindsay ordered equipment to help make her life easier as an amputee. Many of those things had already been delivered and were waiting for us when we got home. I spent some time installing a new shower head with side extensions and will be working on installing and putting together other equipment.

Our life will be different here as the weeks and months go by. Lindsay won't get her permanent prosthetic for a few months and will have to rely on the walker and the wheelchair for the most part. She has to spend time training herself (and her brain) how to do the things she used to do, but doing them without her lower right leg. She still has to keep up on the pain management as well. 

We have received so much help in the past week and will continue to receive and need help in the near future. We'll have doctor appointments, support group appointments,  kids' activities/appointments, etc.

Life goes on. 

We can never really thank everyone for their support that has come in so many forms. We can, however, do our best to rebuild our life...the life we had before the onset of the infection that lasted four years.  

Lindsay's Update...by Lindsay

Sorry for not updating sooner...I've been super busy with different doctors in and out almost constantly, PT/OT work, finally getting some decent sleep, and having lots of visitors (thank you - I LOVE seeing people!❤️). I've finally turned the corner managing the crazy pain after the surgery (not even needing IV pain meds anymore!), and have become a lot more active...practicing getting dressed, walking around the unit using a walker, etc. Also, I just received the pathology report from the amputation, and the incision site showed NO sign of infection, so it has been eliminated!!! (I cried tears of joy ☺️)

This morning I was approved to go to Marianjoy Rehabilitation Hospital (part of Northwestern Medicine) in Wheaton for the next two weeks. I will most likely be transferring there tomorrow afternoon (Wednesday). I've met with a couple doctors/team members from Marianjoy, and they will be working me hard (good!!) for "pre-prosthetic" training (they said that walking with a prosthetic leg will take about 40% more energy than with two healthy legs, so I need to build up some serious strength & endurance, and work a lot on increasing balance). I was told there is no such thing as "over-doing it" with an amputation...the more you do, the faster you heal (music to my ears) 👍 I will have around 3 ½ hours of therapy per day (PT, OT, plus other therapies to help teach my brain that my lower leg is no longer there - this will help with the very bizarre "phantom pain"). Other than that, it will be like any typical hospital (I'll have my hospital room, be monitored by doctors & nurses, medications managed, etc.). But I can also have visitors, and things will be more consistent since I'll have a therapy "schedule," so feel free to come say "hi" (I miss the world)!! 👋

In about 2-3 weeks, I should be able to have the staples removed from the amputation incision, and assuming the swelling has gone down enough, I can start the fitting process for my first "temporary" prosthesis. Then there will be more therapies to learn how to walk/be active with an artificial leg. Once I've gotten that down, I'll be fitted for my permanent prosthetic leg (super customized for my needs). After that, watch out world!!! 😊🏃🏼‍♀️🚴🏼‍♀️🧗🏼‍♀️🏊🏻‍♀️🏌🏼‍♀️🤸🏼‍♀️👍

Anyway, thank you ALL again (and again, and again!) for so much incredible support, kind thoughts & words, and prayers! YOU have been helping me (and my family) get through this challenging time. And thank you so much to everyone who has provided meals, gift cards, donations, help at our house or with the kids, etc. (you know who you are, as do we). I wish I could send out individual "thank you's" to all of you, but the support has been so unbelievable, I can't even begin to keep up. So THANK YOU all!! 😊❤️🙏

Ava's Flower...by Michael

Ava is 11 years young. A strikingly beautiful girl with hair long past her shoulders and wide brown eyes seen through her glasses. She has a laugh that spreads joy and always tries to find the humor in everything. She's confident, strong, and resilient. She's much like a flower, though...she's fragile.

Ever since Lindsay went into the hospital to have her right lower leg amputated Ava's been tight-lipped. She is intolerant of her brother and sister (more than usual) and recoils when family time is mentioned (which is unlike her). She eats less than usual and that beautiful smile has been replaced by a frown.

On Mother's Day, I took the kids to Dunkin' Donuts. Henry and Ella ate their donuts and sandwiches without missing a beat. Ava let hers sit in front of her as she gazed out the window. This is not like her at all as she loves sweets! She didn't want to participate in the conversation at hand. She didn't want a hug or even my hand rubbing on her back. She wanted to go home. After Dunkin' Donuts, we went across the street to the Jewel Food Store where I had some banking to do and then go shopping for a Mother's Day gift for Lindsay. Ava was impatient and wanted to get in line and then go home. She was distracted and painfully ambivalent. 

Our plan was to buy Lindsay a gift (Fannie Mae chocolates) and then go to the hospital to deliver them to her. Ava sat slumped in the car not engaging at all with Henry or Ella...and if there was any engagement it was all negative towards them. She was bitter and impatient.

We arrived at the hospital mid-afternoon and Lindsay was awake when we got there. Henry ran up to her and gave her a hug in her bed and Ella wheeled as close as she could to give hugs as well. Ava stood back and had to be called over for the hug. She was timid, shy, and uncharacteristically quiet. When the hug was over she retreated to the couch across the room and opened up her iPad. That was all we heard from Ava that afternoon. Every so often I would look over to her and I could see tears welling up in her eyes; but she refused to cry or let anyone know they were there.

When we got home Ava asked me if she could stay home from school on Monday. I talked with Lindsay about it and we agreed that she might need that time to continue processing what's going on. Ava told me she was stressed about everything and couldn't put words to her feelings. When I told her she could stay home, a flash of relief washed over her face and she let out a sincere "Thank you."

The next morning I got Henry and Ella off to school. I then mowed the lawn. By the time I was finished and walked into the house I found Ava on the big chair with her iPad, curled up with the dogs. I called her over to me on the couch. Immediately she curled up next to me and laid her head on my chest. We sat in silence for a few moments and I asked her how her stress level was. She replied that it was still there. We talked a bit about how things will be different around home but that this amputation will give her mom the life she's always wanted back...the life of taking care of her family without physical obstacles. It will be hard at times, but it's all for the best.

I thought maybe she and I could go to the hospital on our own and she could have one-on-one time with her mommy. She thought that was an ok idea but then asked if she could FaceTime her instead. I spoke with Lindsay and she thought that'd be great. I took Ava out to lunch and when we returned she dialed Lindsay's number for a FaceTime session. I left them alone to talk.

When I returned and she was finished with the conversation I asked her how it went. She told me it was really good and she sported a smile on her beautiful face with two thumbs up. 

Even though Ava is the oldest she's still a child...our child. And she needs her mother just as much as Henry and Ella need her, if not more. The bond she's created with her mother dictates an ongoing connection that is threatened by Lindsay being in the hospital and suffering. Ava's mind reacted with stress although she couldn't define what emotions were trying to be expressed. It's a complicated twist of feelings and needs. 

Spending alone time with her mother, even through FaceTime, proved to be a blessing for Ava...and one for Lindsay as well.

Maybe Ava's flower will now bloom again.

The Best Is Yet To Come...by Michael

Six of us (me, the three kids, and two grandparents) sit anxiously in the surgical waiting room. Lindsay had her lower right leg amputated due to a four year battle with a foot infection. The surgery is finished and I had already talked with the doctor...we're just waiting for her to come out of the recovery room. Ella sits at a table coloring a picture for her mother. Henry is doing a puzzle book, and Ava is on her iPad. I talk with Grandma and Grandpa. We wait.

The call for Lindsay's family rings out over the people sitting in the waiting room. We gather our things and follow a staff member through the winding halls of the huge hospital. We finally get to Lindsay's room and brace ourselves for what comes next. Ava, Henry and Ella are excited to see their mother but they're unsure of what to expect in terms of what her leg will look like...they have no reference point with this, no experience.

As we enter the room Lindsay is lying almost flat with her eyes closed. She hears our talking as we enter and turns her head toward us. I go into the room first and find my way to the left side of the bed. Ella finds her way to the right side and Ava and Henry file in at the foot of the bed. Lindsay's eyes tell us that she's in pain but also speak of how glad she is to see her family gathered around her. She lifts her hand toward me and I take it into my own. I ask her how she is doing and she moans a bit and says it hurts.

Ella has the picture that she colored for her mom. It is a picture with the words, "The Best Is Yet To Come" surrounded by flowers. She says, "Mommy, I brought you a picture."

"Oh, let me see." responds Lindsay.

Ella hands her the picture and Lindsay thanks her with a kiss to her hand. She gives me the picture to place somewhere and I put it at the foot of her bed where her right foot used to be, so that when she looks over there she'll see the beautiful colors and the all important message it brings.

Lindsay asks the kids if they want to see the stump of her leg. They say a wary "yes" and all heads turn towards the foot of the bed. I grab hold of the blanket that's covering her stump and slowly pull it back, exposing her leg. The kids' eyes widen as they take in the sight; their heads turn a bit as they gather in the reality of what their mother had just gone through. After a minute or two I place the blanket back over her leg and all eyes turn towards her eyes. 

"It's going to be OK" says one of them. Lindsay winces in pain again and a nurse comes in with medication. We all step back from the bed and let Lindsay and her nurse do their thing. It is decided that Grandma and Grandpa will take the kids to get a bite to eat. They leave the room and Lindsay and I are alone.

Lindsay looks at me and asks, "What'dya think?"

I pause for a moment, take the cover off her right leg again and breathe in a sigh. "I love YOU." was my response and I put the covers over the stump again.

We sit in silence for a bit. I then begin to tell her what the doctor told me about the surgery...how everything went well and how everything looked good. This brought some relief to Lindsay...as she's battled these infections for four years now. "Things looking good" was good for her to hear.

When the kids came back they had a "Get Well" balloon that they put at the foot of the bed next to the coloring page placed there earlier. We all stood around Lindsay trying to make small talk and then realized that she simply needed time to rest. Grandma and Grandpa took the kids out and headed home. Upon their leaving the kids looked back at their mother and said, "Goodbye mommy...we love you."

"I love you too" Lindsay replied.

The road ahead will be filled with challenges for us. The road ahead promises to be filled with hurdles we must overcome. The road ahead is not only filled with healing from an amputation but still filled with caring for a child with SMA and two other children as well.

We are fortunate to be surrounded by people who have helped and others willing to help as we navigate this chapter of our lives...and we thank so many people for their love and care. We are blessed to have so many prayers from so many people and so many good thoughts coming our way. 

We take one day at a time, one hour at a time...we heal, we persevere, and we do what we have to do in order to make it through each day.

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Care Calendar information for those who want to help with meals, transportation, etc.:

www.carecalendar.org

Calendar ID: 281993

Security Code: 3296

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GoFundMe Campaign set up by friends to offset medical expenses related to Lindsay's amputation:

Direct Link:

The Casten Family

Copy and Paste Link:

https://www.gofundme.com/the-casten-family?sharetype=teams&member=2200528&rcid=r01-155768444534-eff6ff5da5ec41af&pc=ot_co_campmgmt_w