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.

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...

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---

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. 

DONATE HERE:  https://www.gofundme.com/ellaswheelchair

Wheelchair & Smart Drive : $9890.85 

Thank you,

With Love...the Casten Family

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...

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:  http://mrcasten.wixsite.com/key-to-learning).

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?!

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.