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:  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?!

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".

Wait.

What??

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

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

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

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

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

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

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

Click here to read the Cure SMA article!


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 antibiotics...it had been a very stressful week knowing that I wasn't getting the medicine to help fight this infection and prevent it from spreading to the hardware or my bones.

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

However, our moods went a bit downhill yesterday. 

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

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

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

We're discouraged.

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

We're scared. 

Tuesday, November 8, 2016

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

Well...

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

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

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


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

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



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

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

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

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

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

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

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

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

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

Friday, November 4, 2016

My Foot (by Lindsay)

An update on my right foot...

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





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

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

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

Then it got worse.

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

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

Infection.

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

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

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

Wet to Dry dressing materials

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

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

Ugh. 

Monday, September 26, 2016

Surgery Tomorrow (by Lindsay)

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

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

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

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

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

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

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

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

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

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

Calendar ID: 243115
Security Code: 1914

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

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