Thursday, January 4, 2018

Turning Ella's Corner...(by Michael)

A corner has been turned and Ella's on the "straight" path to recovery. Her posture looks incredible as she sits in her wheelchair.

Before this post goes any further it's important to us to send out a thank you to all of you who prayed for, thought of, and supported Ella throughout the past month...there is truly power there.

Since we  have been home the progression of her healing has been astonishing to watch. Upon arrival in Naperville every movement of hers was filled with pain from some part of her frail body. Sleeping, moving, changing diapers, and bathing took 3-4 times as long as they usually would have had there not been a surgery. Keeping up with the medication regiment was imperative yet came at a price...constipation...which of course has its own remedy; this remedy was fully implemented. 

Each day we saw gains and losses in terms of how she felt and her pain level...two steps forward and one step back seemed to be the call. Amidst a lot of screaming and crying we managed to get through our days in time to prepare for the nights. Toleration of overnight liquid feeds, turning for optimum positioning, and changing soiled diapers became the protocol each night. Not to mention being woken by an alarm to administer overnight meds. 

The corner she turned was never really in sight; the one that got the ball rolling for a smoother recovery...a predictable recovery---a recovery that took one step forward and had no steps back. Toileting became easier, underwear as opposed to diapers entered the scene; toleration of overnight feeds brought much needed nutrition to her body and her fight. Sleeping through the night gave her body a chance to heal and supplied her with the energy for the next day in order to participate in family fun. 

She enjoyed spending time with family over the holidays. She beamed with pride when family members commented on her physical stature--namely straight! She loved the presents she got and always kept the real meaning of Christmas close to her heart. 

I truly believe it was the time she had with family and friends, alongside the pouring out of thoughts, prayers and support that is responsible for a recovery such as this...

Thank you for turning Ella's corner with us...

Ella...23 days after spine surgery

Thursday, December 14, 2017

Spine Surgery Update (12-14 @ 4:30pm)

Overnight (Wednesday -Thursday):

A night filled with some rest, times of turning from side to side, and complaints of pain in the tummy as well as the back. Finding a comfortable position was challenging, but throughout the night we managed to find positions that worked for a short while so Ella could sleep.

Thursday, 12-14-17:

Today was moving day. Ella was transferred from the PICU to the Neurology Floor. With all of her machines and me following behind with her wheelchair, we made the trek from the 16th floor to the 19th. We were no more than in the room and hooked up when the Orthotics Team came to make a casting from her in order to make her specialized TLSO (body brace). The team slipped a cloth onto her like one might slip a cloth onto a rolling pin. The cloth encased Ella from the neck down right past her hips. Then they applied casting gauze; manipulating her body and pushing on the bed as they rolled it under her back. We waited a few minutes as the cast hardened and then they made two cuts down opposite sides of the casting and removed it. All the while Ella screamed and cried. She stopped instantly once it was over...her fear of pain has increased these past days.

The current medication regiment hasn't been working that well so some changes have been implemented. They have tapered back on the narcotics (which make her tummy hurt) and began a regiment of alternating IV Tylenol and Toradol (an IV version of Motrin). The Valium will remain for two reasons--pain and anxiety.  

Her nutrition thus far has been through TPN (nutrition going directly into the bloodstream). Tonight, we started g-tube feeding with just Pedialyte. They started at a low rate and will be increasing the rate by +10 ml/hr until it reaches 75 ml/hr while simoultaneously decreasing the TPN rate. Tomorrow we hope to start her on her regular formula.

A smile ran across Ella's face right before the Orthotics Team arrived as she saw Grandma enter the room. A little Grandma time is always good for the soul. 

A rough morning has given way to a calm evening as Ella has slept much of the day away.

Wednesday, December 13, 2017

Spine Surgery Update (12-13 @ 1:00pm)

12-13-17 @ 1:00 pm:

No position is comfortable. No words are reassuring. No relief has been reached. We traverse on the first 24 hours after surgery and pain management has taken a downward spiral.  

Ella will remain in the PICU for another 24 hours. New meds and an increase in current med doses, change in rates, and tweaks of frequencies have been ordered by her pain management team. They are paying close attention to her hemoglobin as those numbers have dropped while at the same time monitoring her tachycardia (she has an increased heart rate alongside low blood pressure). With all the increases in meds and new ones introduced her respiration has slowed down a bit so we watch that as well. She has gotten very little sleep, has eaten nothing, and is allowed only small amounts of water.  

This has been, without a doubt, a very trying time for us. It's difficult, to say the least, not to be able to provide comfort for her especially because she is asking for it on an almost constant basis. We do all we can for her but nothing seems to work...hopefully, the new regiment of medications will allow her some relief. 

We know many of you have prayed and sent positive thoughts and support our way; and for that we are grateful. 

It's times like this that we so deeply depend on our family and friends...

Thursday, August 17, 2017

Ella's Smart Drive Wheelchair

Through the generosity, encouragement, and support of so many people we were able to purchase Ella her new wheelchair that will stay with her for many years to come. Currently she is in the practice stage of this wheelchair but she is doing great! We are so very grateful that we have such a great community of friends and family surrounding Ella.  Thank you all...

Take a peek---

Saturday, July 22, 2017

New Possibilities with a New Wheelchair...(by Michael & Lindsay)

As Ella grows older her needs change, mature, and expand. She’s at an age where she wants desperately to explore her world yet access to that world is hindered by SMA. Her power wheelchair gets her around most places but also confines her to certain places. She very rarely, if ever, goes to a friend’s house to play because of her power wheelchair. Just getting it into a house can be an ordeal. Even when friends come over to our house they have to stay on the first floor. Ella has a small manual wheelchair but she has outgrown it and she also cannot move it over carpeted areas (like our basement). 

Just recently we discovered a lightweight, collapsible manual wheelchair called the "Zippee Zone" with a separate device with a power wheel that can attach to the back of the wheelchair to move the wheelchair along (called a “Smart Drive”). Unfortunately, insurance will not help pay for this chair, as they pay for her power wheelchair. If we purchased this manual wheelchair and device for her she would have a wheelchair to use upstairs, down the basement, at friends’ houses, and as a back up to her power chair in case it has a problem. The “Smart Drive” uses a wristband remote control so that when Ella taps her wrist on the wheelchair’s wheel the Smart Drive moves the wheelchair forward for her. She turns it like she would if she were powering it herself.  She would also have complete control over acceleration and other aspects of the Smart Drive through the programmable wristband. This would open up a whole new world of possibilities for Ella.

She could visit anyone’s house as the chair would tie down in the van for transportation purposes. It only weighs 15 lbs so it’s quite portable. She could use it in the basement, on carpeted areas, upstairs...anywhere really. She has already tried out a demo model and she loved it. So did we. 

Please consider helping us purchase this wheelchair and the Smart Drive for Ella with a donation of any size. 

Wheelchair & Smart Drive : $9890.85 

Thank you,
With Love...the Casten Family

Monday, May 1, 2017

Dare We Say?...(by Michael)

Two weeks sounds like an eternity. Two weeks seems so far off.  Two weeks seems untouchable. Ella's first Spinraza dose is scheduled for two weeks from now!! YES--insurance gave her their final approval!!

A treatment, that in our minds was far from our grasp; yet we sit in the kitchen talking about what that first injection will be like; we talk about what to expect, how to handle any pain, and ways to be realistic about waiting to see what happens next.

In the art of writing there is always the author's intention to lead to a climax, a heightened awareness of what the story is all about.  You would think that this news would be the climax of the "Ella Story"---but dare we say,  dare we venture to ascertain,  dare we challenge the notion...that this is not the climax? This is not the high point for Ella? That this is just the beginning of new and exhilarating chapters in her life? 

We hold hope for her---hope for wonderful things yet to come.  

Let's watch and find out...

Friday, March 31, 2017

Added to Our Plate?! (by Michael)

I sit in a familiar place...Easter Seals...for Ella's Physical Therapy.  She sits on the floor working hard on her therapy, making the best of what she has...or better put---what she has left.  SMA is relentless as it robs its victims of what they have...that's why the entire SMA Community is so eager to get their child (with SMA) approved for the new treatment drug known as Spinraza.  It's proving, however, to be an uphill battle with insurance companies.

Yesterday I spent the entire day with Ella at Lurie's Children's Hospital for her "clinic".  This is where we meet with her team of doctors and therapists every 6 months or so; a team that is dedicated to their patients in so many ways.  Ella's visit went wonderfully.  She's gaining weight, growing, and maturing.  She performed well on her pulmonary tests and completed her first DEXA  Scan (bone density) which set a baseline for her.  The only part of the day that elicited anxiety for Ella was the unexpected blood draw.  This was ordered in the hopes that Ella will soon be able to receive the new SMA drug, Spinraza.  Her team wants her to be ready just in case she is approved.

While at "clinic" the conversation quickly went toward the progress of Ella's request to the insurance companies for Spinraza.  Her team told us that all the necessary paperwork on their end has been submitted to our medical group and once the referral is approved by the medical group the submission will then go to our Blue Cross Blue Shield (BCBS) Insurance Company.  It's a slow process but there is progress being made in terms of getting through the red tape.  We are so grateful for those who work diligently behind the scenes to help things move along.

In the meantime, we have a busy summer coming up.  In addition to Potter (our 11-month-old Vizsla) we just recently added a new member to the canine side of our family!  It's a pleasure to introduce Ginny (Potter's 9-week-old sister...same parents)!  So our plate will be graciously full with two young dogs racing around the yard and going to the dog park on an almost daily basis; full with taking care of the kids' needs including soccer, baseball, swimming, piano and violin; full with getting Ella back and forth to her various therapies; full with Lindsay's work at church getting Vacation Bible School (VBS) up and running, and then turning around and preparing for the fall Sunday School, LOGOS, and Milestone programs; full with a planned trip to Leesburg, VA, to visit with Lindsay's sister and the entire Gibson family (our dogs included!); and full with my summer tutoring program (Casten's Key to Learning) which already has five students (please feel free to pass along the following website if you know of anyone needing summer tutoring--both in-person and online sessions available:

Our hope is that one more great thing will be added to our busy plate...a summer with Spinraza. 

One of Ella's Pulmonary Tests
Ella's First DEXA Scan

The "Kids"

Potter & Ginny getting trouble?!

Tuesday, January 3, 2017

Answered Questions... (by Michael & Lindsay)

Since we last posted the incredible news about the FDA approval of the first treatment for SMA several people have asked some great questions that we'd like to share the answers to...

This is a treatment and not a cure for SMA.  It allows the back-up gene(s) the ability to produce more full functioning proteins needed to keep existing motor neurons alive.

The treatment cannot reverse any damage that has already been done.  It cannot inspire growth of new neurons either; nor does it increase strength on its own.  

The idea behind this treatment is that it could potentially slow down the progression of the disease. It is not designed to stop the progression of the disease. 

Many kids during the trials showed marked improvements in motor functioning while some showed less. This tells us the drugs works (or doesn't work) on a case by case basis.  

Once we go through the red tape and Ella is taking the treatment will we truly know if this is something that will benefit her...what happens from there is yet to be seen.

We are hopeful that Ella will benefit from this treatment...hopeful that all people with SMA will benefit.

Friday, December 23, 2016

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".



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 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 is actually here.

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

Click here to read the Cure SMA article!

Saturday, November 19, 2016

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 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.