"Back in the Saddle Again"...(by Michael)

The calls for fairness abound since Ella's surgery.  

She is realizing and expressing her frustration associated with the confining nature of not only her hip surgery, but with SMA in general.  Much too much for a five-and-a half year old to have to endure. Cries of "It's not fair" have resonated throughout the house.  And she's right...it isn't fair.

Before the surgery, Ella had never really claimed that life was unfair.  She gracefully accepted her limitations and often astounded us at her resilience, adaptive nature, and positive outlook on life.  When things seemingly were out of her reach she suggested a way to accomplish her goal in a modified way.  She has taught us that if you simply step back, take a moment to see the situation for what it is, take what you already have, and modify it to allow her access--then she can participate.  One of our goals in this healing process is for her to fully return to that inspired and wise child.

Ava had a friend over this past week.  All three kids like to play together when this friend is over. The four kids were sitting in the living room.  Ava, Henry and the friend were sitting on the couch and Ella was confined to her adaptive medical chair.  They were deciding what to do.  Ella quickly realized that the other three would soon be moving about...from room to room or from inside to outside.  The protests began.

"It's not fair" Ella claimed, "they get to move around and I won't be able to play; no one will push me and even if they did, that's no fun."

The other kids, who usually are able to figure out a way for Ella to play, were dumbfounded.  Nonetheless they wanted to play by moving around, not sitting idle.  The dilemma grew into arguing and fighting.  Eventually Henry left.  Ava and her friend stuck around for a while, maybe 5 minutes, until they decided to go to the friend's house instead.  Ella, still in the same spot from when the friend arrived, was silent.  She was abandoned and there was nothing she could do.  She couldn't move herself, she couldn't advocate for herself, she couldn't participate.  She sat in silent contemplation, most likely thinking about how SMA has limited her.

I sat and watched this happen.  My heart broke for Ella.  It broke for all of the kids because I knew how much they loved playing together (even though they fight at times!).  I felt the frustration, the anger, and the destitution as a result of SMA's intrusion on their collective childhoods.  I breathed deeply to myself, trying to thwart the emotions that seep in each and every day...the emotions that want me to cry out, "SMA sucks.  SMA is a terrible disease that no one should have to endure.  SMA has robbed my family of so much."  I was faced with a choice.  I could allow the negative emotions that infiltrate our household once again take hold of me or I could use what God has given me and find a way to make things better.  

Ella sat in silence.  The room was quiet.  I looked around, hoping that something would come to me. I spotted Ella's power wheelchair.  It hadn't moved for weeks.  As a rolling wave the negative emotions receded from my heart; in its stead an inspired thought entered, filling me with determination and insight. The wheelchair that serves as Ella's physical connection to so many aspects of the world she lives in must be able to be utilized.  The inspiration became determination.  

I stood and went to the wheelchair.  I lifted one of the armrests to explore how it might be adjusted.  Ella is very possessive of her chair and asked what I was doing.  I told her that maybe, just maybe, I could make it so that she could use the chair instead of the medical stroller.  Her eyes lit up. And for the next several hours, as I worked on adjusting heights, angles, and attached equipment--she sat watching, waiting, anticipating, and hoping.     

Lindsay joined me in the endeavor and between the two us we worked our way to a solution.  We made sure safety was a priority and comfort followed suit.  Before the sun went down we were finished and the moment of truth stood before us...staring us in the face.

Placing Ella in the chair provoked anxiety for all.  Her posture was quite different since that last time we placed her in it.  We tinkered a bit with positioning; reassured her that she was safe and secure.  With eagerness and trepidation Ella turned the chair on.  Within 30 seconds she was cruising our hallways and rooms as if she never missed a beat.  We opened the garage door.  Down the ramp she flew and out into the brightness of the day; spinning, racing, and smiling a smile that only a child can produce.  

A sense of satisfaction overwhelmed me.  Yet I knew we weren't alone in this.  God has been so gracious, so important, and so loving...we are blessed beyond measure. This was no different; He was there. 

Ella's spirits rose that day.  Her healing has taken on a new phase.  She said to Lindsay on the way to church, "See Mommy, I feel like everything is normal...I just have a cast on!"

We still have a ways to go...nights are not as rough as they were a week ago but days are great.  We are looking forward to our first follow-up appt. with the surgeon (April 18) to see how healing is progressing.  We still have to keep her on pain medication but that has lessened over time.  All in all, we're on the right path.

We continue to pray for healing, strength, and guidance. God is unfailing in all.  He has placed before us a task to complete. He has placed before us children to care for--surrounded by circumstances not many will experience.  He provides blessings for us to use in order to best care for our family, and at the same time, provides inspiration.  Ours is a road less travelled; a road that often captures our emotions, taking them to extremes.  A road, without question, that leads us back to Him.

"Another One Bites the Dust"...(by Michael)

And so it continues...

Ella is home.  By 7:40 in the morning it's just the two of us, Daddy & Ella.  She's making great progress as her pain has subsided and her spirits have grown.  We've embarked on a joint effort to establish some kind of routine and so far it's working well.

Navigating a "Hip Spica Cast" is not without its challenges.  Everything we do is done in slow motion.  The name of the game is "slow and gentle".  From combing her hair in the morning to re-positioning her at night...slow and gentle.

One area that has been undoubtedly challenging is our nights.  We have had three nurses since November of 2015 and are in the process of getting a fourth one. Our first nurse simply stopped showing up after just two weeks (nice, huh?).  The second nurse was a temporary for the first until the agency could find us a permanent one.  Finally, the third nurse came in January (2016).  

As many of you may know, right from the start it was a rough go with the third nurse. We attributed that to Ella having so many nursing changes in such little time.  As time went on, however, it was apparent that the nurse and Ella just didn't connect; not for lack of trying by all involved, it just didn't happen.  We were really looking forward to having a nurse after the surgery, thinking we had that "base covered".  It was hope against hope. It simply wasn't going any better when we returned home.  In fact, it became worse.  We are so exhausted from having to get up umpteen times over the past few nights. Not to mention Ava and Henry--who were also woken by Ella's hysterical screams of protest; they have actually expressed their utter exhaustion to us.

Kids are in tune with their environment--and Ella's night-time environment wasn't conducive to her (or anybody else's for that matter) getting a good night's sleep.  Besides, if we are getting up more than we were before this nurse (or even before nursing) then what in the world is the point of continuing with her?

We have reached out to the agency to find another nurse.  We know that if we find a nurse Ella will feel comfortable with then it would be just fine.  We told the agency that we'd like for Ella to be a part of the process in choosing her nurse.  The last three nurses barley met Ella before they started working; the system is broken in that respect.

And so it continues...what was said between Lindsay and I when we first learned of Ella's SMA Type 2 diagnosis holds true to this day..."We're not here to save her...we are here to take care of her."

Discharge Day!! (by Lindsay)

Ella is coming home today!!!

When I arrived at the hospital this morning, Ella was in GREAT spirits...talking, smiling, laughing, singing - our typical Squishy!

Yesterday afternoon Ella fell asleep around 2 pm and didn't wake up again until 8 am this morning! 18 HOURS!! I guess she needed it :)

Ella is tolerating her regular formula at the normal rate, she has minimal pain and she is VERY excited to go home!

Ava, Henry and Ella are SO excited to see each other...they have missed each other so much! I can't wait to see the reunion :)

Michael is feeling well rested and has done such a great job of being here and caring for Ella 24/7! He's going to be home with her this next week while I return to work and then the following week is Spring Break for all of us. We're hoping Ella can start returning to school after that!

We cannot even begin to thank all of you enough for your thoughts, kind words and prayers!!! We will continue to update the blog with Ella's progress.

Thumbs up and ready to go home!!

Better Every Day! (by Lindsay)

Overall, Ella has had a really good day!

She slept a lot better last night (including a 5 hour stretch!).

This morning the pain team removed her epidural and her Foley catheter was taken out. Michael and I have successfully changed a wet diaper since then :)

We stopped Ella's IV pain meds (Dilaudid) and switched to oral Oxycodone (in her g-tube) and she's been doing fine with the change!

She's continuing to tolerate her bland formula as we increase the rate. She'll be at her normal rate tomorrow morning. At that point, we'll gradually add the remaining ingredients of her formula, making sure she's not experiencing any nausea or vomiting.

If things to continue to progress in the positive direction like they have today, our plan is for Ella to come home on Sunday!

We know that Ella is really starting to feel better because instead of crying in pain, she's crying over silly things...like when her hair gets stuck behind her neck, or when the Disney Junior channel randomly switches to Jeopardy. That's the 5-year-old we're used to ;)

Ella had several visitors today that made her smile A LOT! She really lights up when some of her favorite people come to see her here :) But she is also excited to be home so she can see Ava, Henry, Sasa and her friends, too!

Ella, you impress us with your strength every single day!  Keep it up, Squishy!!

Our Little Fighter

Ella with Gramma

Ella with Mrs. Newell (her physical therapist at Prairie)

Ella with Mrs. Johnson (her kindergarten teacher) and Mrs. S. (her assistant)

Uncle Andy visited yesterday :)

Yesterday Alli Traven brought an AWESOME gift from the 4th grade team at Lincoln (Michael's school)...4 different nightgowns for Ella (she can't wear pants with her cast) each with a matching one for her baby!!

Making Progress (by Lindsay)

Slowly but surely, Ella is making some progress.

Last night, she was able to sleep for stretches of 1 to 2 hours at a time.

However, she continued to be in a lot of pain this morning.

Here is where we are with pain management:

• We met with Dr. Grayhack and he ordered yet another IV pain med, Toradol. We think it might be helping!
• We stopped the analgesic (nerve-block) in her epidural, but we are keeping the actual epidural in her back. Once we see that she is ok without the analgesic, we will remove the epidural and urine catheter (currently planning for this to happen tomorrow morning).
• Tomorrow afternoon, we will try switching from the IV Dilaudid to oral Oxycodone (in her g-tube), both strong narcotics.
• In order to go home, we need to see that she can be off all IV pain meds, including the new Toradol that we just added today (only available through an IV).
• She will go home with Oxycodone, Gabapentin (nerve pain med), Valium and Tylenol for pain.

Ella's heart rate has still been consistently very high. It has never dropped below 145, even while sleeping. Hopefully once her pain is under control, we will see these numbers start to drop closer to her normal range.

The other challenge we have to conquer before going home is getting Ella to tolerate her feeds. We have made some definite progress with that today!

• This morning we once again tried a very bland version of her formula at a very slow rate and she has been tolerating it so far!
• Every 4 hours, we will increase the rate by 5mL/hour until we get to her normal rate (hopefully by Saturday).
• If she tolerates the bland formula at her normal rate, we will make her regular formula and give it to her at that rate. We will observe her with this for 24 hours to make sure she can tolerate it (if all goes well, this will bring us to Sunday).

We also have to learn how to change Ella's diapers with her cast (since she can't sit on a toilet with her cast on, she will have to wear diapers for the next 6 weeks). Once her urine catheter comes out (most likely tomorrow), she will start to have wet diapers. We also have to make sure Ella has at least one bowel movement before she goes home. She currently doesn't have much in her stomach and the anesthesia and pain meds make her constipated. Once we start giving Ella her regular formula, she'll be getting Miralax and Colace (stool softener) to help the process along.

While we are not yet in a position to start planning a day for her discharge, we at least know where she needs to be in terms of pain management, feeds and going to the bathroom, and we're moving in the right direction :)

Ella has definitely been in better spirits today, and it is SUCH a relief for us to see. She still whimpers and cries in pain quite a bit, but it's not constant like it has been since the surgery. One thing that has really helped to distract her is having visitors. She even gave us a smile today when she was with her Uncle Noah!! She keeps asking when more people can come :)

We're so glad that Ella seems to be turning a corner. The first 2 to 3 days after a surgery like this are always very hard, but once we figure out the right combination of pain meds and we're able to get some food in her tummy, things start to move in a positive direction!

We cannot thank ALL of you enough for your thoughts, kind words and prayers! It means the world to our family and it's what's keeping us moving forward! Thank you!!

A Rough Road (by Lindsay)

Ella has been having a rough time.

She is extremely uncomfortable. She's hardly been able to sleep. When she does finally drift off, it's because she's worn herself out by crying so hard. Then she'll quickly jerk awake from the pain.

And the cycle continues.

Ella still has a nerve-block being continuously administered through a lumbar epidural. She is on a strong narcotic pain killer in her IV. She's on Tylenol. We added a nerve pain medication. We added Valium.

Nothing is helping to ease her pain.

We increased most of her pain meds. Still not much improvement.

Ella's heart rate has been very high compared to her "normal." And it has been constant. It has been ranging from 145 to 165 (even while asleep), sometimes spiking to 175. Her normal heart rate is under 115 and between 80 and 90 when asleep. Her team explained that the cause for such high numbers is pain.

Ella's IV is causing her quite a bit of discomfort as well. It has been checked multiple times by the IV specialists and it is still in the correct position and is fully functional. The problem is that it is a very large IV (the team refers to it as "the hose") to accommodate all of her meds as well as her TPN (IV nutrition). The pain meds are also very strong, so they are causing irritation to her vein. Her arm has become quite swollen.

Ella is still not tolerating her feeds. We've attempted giving her a very bland, basic version of her typical formula at a very slow rate, but she vomits almost everything that enters her stomach. She's on an anti-nausea medication, but it's not helping. So for now, we have stopped attempting to feed her through her g-tube and she is continuing to receive her nutrition through her IV. We will be increasing the volume tonight.

The original plan was for me to go home this afternoon and take over caring for Ava and Henry and return to work while Michael stayed with Ella 24/7 until discharge. But with Ella's current struggles, I know that I need to be here with Michael, helping Ella get through this. So thanks to the very understanding administration and staff at the Ann Reid Early Childhood Center, as well as the incredibly supportive family members and friends to help with Ava and Henry, I will be taking tomorrow and Friday off to be at the hospital where I'm needed most (however, I will still return home each night).

This afternoon, Ella was moved up to the pulmonary floor...her breathing is stable, which was the risk that had her in the PICU for closer monitoring to start. We are glad to now be on a much quieter floor :) Ella had a visitor today in her new room and it definitely put a (much needed) smile on her face! She is looking forward to several more to come in the next few days! Unfortunately, during cold and flu season, no one under the age of 16 is allowed to visit patients in the hospital, including siblings. Ella misses Ava and Henry terribly, but luckily we have cell phones and Face Time :)  But it will no doubt be a wonderful reunion when Ella comes home :)

Please continue to keep Ella in your prayers...we are very hopeful that she will turn a corner this weekend with her pain and that hopefully she'll start tolerating her feeds! At that point, we'll be able to discuss the time for her to come home :)

Surgery Was a Success! (by Lindsay)

It's been a long day. My alarm went off at 3:45 am.

But of course, Ella was a trooper and full of smiles as usual when we arrived at the hospital :)

Once the doctors and nurses started coming in to talk to us, some anxiety kicked in, so the anesthesiologist gave Ella a little something to calm her nerves...it definitely did the trick!

The surgery lasted about three hours and it went exceptionally well! Dr. Grayhack was able to put the stronger plate and screws in with a little bone from Ella's pelvis for extra stability. He said the screws were holding the plate VERY well :) She will NOT require an additional surgery!!!

Ella had a rough time coming out of anesthesia and was in a lot of pain (mostly her knee). She does not like her cast, even though it is hot pink and her baby doll has a matching one! She keeps asking every nurse and doctor if they can take it off (even if just for a little bit). She will have the cast on for 6 weeks.

Right now Ella has a lumbar epidural in place with a continuous analgesic (nerve block) running as well as two IV pain medications. With these medications, she has been able to drift off a little bit here and there and get some rest :)

Ella is also battling some nausea and vomiting, so she is on an anti-nausea medication. We started a very basic formula through her g-tube a little while ago (at a very slow rate), but she is still unable to even keep water down so we are also going to be giving her some nutrition through her IV.

Currently, the plan is for Ella to stay in the PICU for a couple of days and then move up to the pulmonary floor and go from there. We're not yet sure how long she will be staying in the hospital, but we'd love for her to be able to come home sometime this weekend.

We're hoping she's able to get some sleep tonight and that we're able to effectively manage her pain and nausea/vomiting moving forward.

We will continue to update Ella's blog with her progress :)

Thank you so much for all of your kind words, thoughts and prayers for our Squishy!! We could never do this without ALL of you!!

Take Two...(by Michael & Lindsay)

As many of you already know Ella had bi-lateral hip surgery with some reconstruction of the left socket this past summer.  
Since Ella never walked her hips did not develop normally--the head and neck of the femur bone should develop a nice socket in the hip bone as babies grow into toddlers and young children.  The head of the femur should rest deeply in that socket.  Bearing weight by walking allows not only the angle of the neck of the femur to be formed into an approximate angle of about 120 degrees to the long bone but also hardens the all the bones.

Ella's angle was almost 180 degrees and definitely not in the sockets...in fact, the left socket was barely developed (hence the reconstruction part of the surgery).  Since the degree between the long bone and the neck of the femur was practically a straight line the entire right and left femur bone began riding up rather than angling in.  Her alignment was way out of whack and as it progressed her sitting balance was compromised.  Needless to say it would have only become worse and more painful over time. 

During the surgery the right leg went beautifully and as we look at it now it is fantastic!  Wonderful range of motion and the x-rays show a hip joint that looks like a hip joint should. The left leg, however ran into some complications.  During surgery when the doctor was placing the bracket into the neck of the femur the bone, compromised in strength by SMA, broke.  He placed the bracket in a slightly different position and we waited for bone growth to take place.  During the past few months the bracket has slowly been slipping out. 

Ella's in pain.  

Ella's hip x-rays (March 2016)

Corrective surgery is scheduled for next Tuesday March 15, 2016. For this surgery the new bracket is a different type that has more screws which are also longer going into the long bone and the neck of the femur (the above bracket is only screwed to the long bone).  With that being said the surgeon will have to determine if the bone itself is healed enough and strong enough to take this new bracket. That determination will be made during this upcoming surgery. 

Here's the status after consulting with her surgeon a few days ago:

If her bones ARE strong enough for the new bracket/screws:

• The surgery will last about 3 hours
• He thinks she will stay in the hospital 2-3 days, starting in the PICU and going from there (last time he estimated 3-4 days and it ended up being 7)
• She will be flat in a plaster body cast for 6 weeks to prevent ANY movement (there is a slight chance that she can be in a removable body splint like last time, but he thinks he'd prefer that she is unable to move that area at all)
• She might be able to start easing back into school 3 weeks after surgery (after Spring Break), if she can tolerate it (starting with half days). 
• She will be in the reclining medical stroller and will need someone to push her. She will also be in diapers, as she won't be able to sit on a toilet.
• After the cast is removed, she can slowly start to move into an upright sitting position (will take weeks before she will be able to sit in her power chair).
• He hopes she will be fully recovered 8-12 weeks after the surgery (resume sitting in her power chair, stander, etc.).

If her bones are NOT strong enough for the new bracket/screws:

• He will take some bone from a different area of her pelvis and insert it into her hip area to make it stronger.
• She will be in a plaster cast for 6 weeks while the area heals.
• She will then go back and have the bracket surgery (and start over at the first bullet point listed above).
• It may be 5 months before she is fully recovered.

We know that correct hip alignment will be the best thing for Ella as she gets older.  We know that if we had waited to do the original surgery it would have been much more risky to do later since the disease leaves her weaker and weaker over time.  We know that the pain she feels from surgery will eventually go away, but the pain from the femurs being out of their respective sockets is excruciating and that pain lasts forever unless fixed.  We know her doctor is top-notch in the SMA world.  We know that some circumstances beyond anyone's control led to some complications.  We also know that the consequences of the complications are able to be addressed--and we're grateful for that.  

So we step back.  We take a deep breath.  We once again do whatever we have to in order to take care of our Ella.