25 Days

IMG_20160304_202202498

In 25 days, Emmie will have a CT scan.  After the CT scan, Dr. Chern will make the decision.  If her bones are grafting to the fusion, then she’ll be taken to the special procedures room and the halo will be removed.  If they aren’t grafting, she’ll wear the halo a little longer and then we’ll re-evaluate.

In 25 days, we might be able to cradle our daughter close to us again.  To feel her cheek against us.  I still can’t imagine what that will be like.

She’ll wear her pre-halo brace for a little while and clothes.  Clothes!  Dresses, shirts and pretty much anything cute I can squeeze over that brace.

Please pray for us and Emmie.  Pray that the bones are grafting.  Pray that the bones are strong enough to support her head.  Pray that the surgery will do what it should have done…made her neck straight.  Pray that we can trust God and not “mother hen” her until she is annoyed with us.

So, here we go.  Counting down with a real live date on the calendar.  We have our counting chain made and are ready to start ripping those rings off!

Facebooktwittermail

Hospital Days Pt 2

Emmie slept most of the day on Thursday.  She had to have a little oxygen after the vent came out but that was only for a couple of hours and it was a very low flow.  When we moved out of PICU Friday, we got to really see her come back around.  She saw her toys and wanted to play.12755047_10208641503178739_1848984737_o
And, of course, go for a walk.IMG_20160219_161001117

The kids all came for a visit on Saturday and Emmie couldn’t have been more excited to see them.

IMG_20160219_161634625 The beautiful weather meant a trip outside. IMG_20160220_134942760 IMG_20160220_141133271

Emmie enjoyed watching a construction crane in the parking lot.IMG_20160220_140055155 IMG_20160220_135201406_HDR IMG_2919

IMG_2920

Grandmere and Papa were awesome and so willing to help.  They got to watch the kids for a few days and then Ge and Granddaddy got the same privilege (at least I hope they think it was a privilege).

IMG_20160222_085357097

Our first happy time in the therapy chair was on Monday morning.IMG_20160223_120423070

Emmie’s room.  All cleaned up and ready for us to head out.

IMG_20160223_130507

She broke out on Tuesday right as we got there and discovered that the parking lot was full. No worries as I circled back around and she got valet treatment.
IMG_20160223_130027300

Our traditional meal at Chick-fil-a after discharge.  She loves the chicken nuggets.

Facebooktwittermail

Hospital Days Ahead Pt 1

The day before surgery, we took off to Atlanta for Emmie to have her pre-op appointment.
IMG_20160216_123730137

Mark and I were in awe.  She walked right up to the scale, allowed Mark to help her up and stood there while it took her weight.  Then off she traipsed to the room.

IMG_20160216_123951796_HDRWhere she lifted up her leg for the blood pressure cuff and stuck out her finger for the pulse ox.  Not a single whimper or cry.  That was a first.  She knows the drill now.  We’re so excited that the stresses of a normal vital check are over but so sad to know that she has gotten used to this and it’s just a part of her life.IMG_20160216_124439082

The fun little cozy coupe did not hurt her calm demeanor at all.

After a quick blood draw we were off to have some fun.  We went to a local mall that had an American Girl store and a carousel.  Both were big hits!

12744040_971356672957814_6174094060143595552_nA sweet blessing at the American Girl store came as we spoke with one of the sales clerks.  She wanted to know all about Emmie.  I mentioned she was having surgery the next day and we wanted to do something special before then.  Emmie spent the whole time playing with the babies and the play food.  I talked Mark in to getting a diaper bag for her (it came with goodies, ya’ll).  As we were picking out stuff for it, the store manager came over with a bag of stickers, coloring sheets and all sorts of prizes.  I felt bad because all Emmie wanted to do was play with the babies at the time.  She definitely has enjoyed those goodies since then and American Girl made sure to win us over even more with their sweet sales staff!IMG_20160216_164519528
IMG_20160216_155452468

Emmie loved the carousel but didn’t want to ride the horses.  Just give her a bench to sit in and go around and around on and she’s happy.

IMG_20160216_174347922

We then went out for supper at a much recommended restaurant.  It was pretty good.  This was our (Mark and I) Valentine’s day celebration.  Emmie was so sweet and content to eat her fruit and fries while Mark and I chatted away.  Okay, we chatted with her too.

 

We have family of family that lived near by and had offered a long time ago to allow us to crash at their carriage house if we ever needed to.  We decided to take them up on the offer for this trip.  It was so nice and quiet and perfect for a quick rest before heading to the hospital the next day.  This is what she did when she realized she was going to share our bed.IMG_20160216_204245368

After Emmie and Mark got a good night’s rest (nerves and prayers woke me up at 2:45 a.m.), we headed to the hospital. IMG_20160217_061156111 Papa and Grandmere made a super early morning trek to be there before surgery.IMG_20160217_055648421

I realized in the waiting room that we had not gotten a picture of her curls and I snapped one really fast knowing they may all be gone by the end of the day (they had to cut/shave the bottom half of her curls).

IMG_20160217_063658609 In our personal surgery room just waiting.  She did good again with the blood pressure cuff and pulse ox.  IMG_20160217_070445449_HDR After her happy juice.  She was as good as can be.IMG_2906 The anesthesiologist came by and talked with Super Bear.IMG_2905

Grandmere and Daddy just hanging out.

IMG_20160217_152432059

Let the waiting begin.  This was after lunch as we watched the surgery waiting room slowly clear out until only us and an emergency surgery family were left.

12745619_971691622924319_7675328260496025913_n Meanwhile back home…The kids had gotten the shirts in on Tuesday and were ready to show their support on surgery date.  Aunt Tracy came by to get her shirt as well.12745600_971691636257651_3875258617570727780_n 12733365_971691666257648_4096551291608311270_n That right there is a good friend.  Braving my children, her children and a few extra while unknowingly preparing to deal with a stomach bug after she left (three of her kids and herself got it…only one of ours did).10414570_971691679590980_3495728045918124308_n

IMG_20160217_110519153

Ge and I checked out the garden.  And Ge promptly cleaned out the fountain.  It was cold ya’ll.  But she said the pump would get clogged and it needed to be done.  Anything to keep busy, right?

IMG_20160217_204926680

Then time passed and we had her.  We were with our girl.  I was not prepared for how hard it would be on her.  The silent cries as she tried to tell us she was hurting and uncomfortable.  We know the ventilator was necessary for her protection but we were so glad when it came out.  We were told she would be sedated and they tried.  Oh how they tried.  But, the forced air from the ventilator kept waking her up and making her uncomfortable.  They had her on three to four different sedation meds and she never could get in to a good sleep until the respiratory therapist turned off the ventilator at 4 am.  Then sleep.  Glorious sleep. She rested so well until morning rounds when they removed the ventilator.

IMG_20160218_093553410

Then we got to hold her and let her sleep some more.

IMG_20160218_190421454_HDR

That evening, she got to see her brothers and sisters on skype.  I know it doesn’t look like much here but her whole attitude changed once she saw her sidekicks.  She was thrilled to see them all.

Facebooktwittermail

The First

Emmie had spinal fusion surgery on February 17th.  Remember when I said that NF was a marathon?  February 17th was one of the longest miles.  She went back with her neurosurgeon at 7:30 in the morning and we saw her at 8 that evening.

The logistics went like this:  They went in to the front and took out two vertebrae.  Then they stitched her up, flipped her (with the halo for stability, breathing tube in place, etc).  They put in metal rods in the back of her neck, secured with hooks.  Secured with hooks because they don’t make screws small enough for her bones.  Then they closed her up, flipped her.  Again.  And replaced the two previously removed vertebrae with a straw filled with her bone.  She spent 24 hours on a ventilator and only had a slight dip in her breathing when it was removed.  She was on narcotic pain relievers Wednesday and Thursday but by Friday morning pain was being controlled with acetemetophin only and we were moved to a regular room.  We are also pretty certain that valium makes her throw up.

IMG_2914

Going over the details with Dr. Chern.

To say this surgery was extensive is an understatement.  It was intense and a lot of work and a lot of quick thinking and planning on our neurosurgeon’s part.  He and the whole surgical and orthotic staff were amazing.  Emmie’s orthotist came out each time he was with her to give us a personal update.  Dr. Chern came out of surgery and was so great at going over everything they did and how she did.

They straightened her spine, ya’ll.  It went from this:

cropped to this:  IMG_20150802_193024787in a matter of hours.

She did well in the hospital.  Over the weekend, we were worried because she refused to stand.  When we brought it up with Dr. Chern, he wanted to get some x-rays done and have physical therapy come by.  We were just trying to rule out muscle and neurological issues.  By the time physical therapy came by, she showed off and walked to the bed with no problems.  She continued to improve enough throughout the day for us to go home on Tuesday.  Six days in the hospital.  And then home.

IMG_20160222_084507691

Recovery is going to take some time.  In adults, kyphosis surgery is not nearly as involved as hers had to be and it takes about two to three months to recover.  Six months to fully recover.  But, she’s up and walking.  She’s laughing and talking and this weekend she wanted to climb stairs.  Each day she is getting stronger.  We’re continuing with the over-the-counter meds of ibuprofen and acetemetophin and that keeps her mostly happy and going.

To our knowledge no one has really done spinal fusion for kyphosis on a child under 2 years of age.  She’s one of the firsts.  She was a first for our doctor.  Doctors all over the country are watching and learning from our Emmie.  Whether this is a permanent fix or not, we know that God is using Emmie to help children that come after her.  We can’t wait to see how God is gong to write her testimony (or any of our kids for that matter).

What’s next?

Next comes the biggest step.  Emmie will go back sometime in around two weeks.  She’ll be put under general anesthesia and they’ll do a CT scan to make sure the bone is grafting to the rods and straw.  If that is confirmed, they’ll take her in to the special procedures room and remove her halo.  She’ll wear the neck brace she had before the halo for a few more weeks and then she’ll be brace free.  For the first time in over a year.

And then we watch closely and pray.  If she has spinal stability over the next six months, we can consider the surgery a success and no longer have to worry about kyphosis.

At some point after the halo is removed, she’ll also have an MRI to check on the growth of the plexiform neurofibroma.  We were unable to do it in the halo because it would present with artifacts (distortion) and make it impossible to see what is going on. The rods and plate may cause some distortion but it should not be as bad as when the halo was on.

Was.  I can not imagine life without the halo.  It’s just become a part of our lives.  Part of who she is.  And yet, I can not wait to feel my baby’s cheek against mine.

Facebooktwittermail

The Countdown Begins

February 17th is the day.  At that point, Emmie will have been in the halo for 6 months and will be 21 months old.

We’ll be updating the Facebook page throughout the procedure and the days following, so if you are on Facebook, that will be an easy way to keep up with our prayer requests.

We were so blessed, within an hour of scheduling surgery, our friends stepped up to provide childcare.   This is a big blessing so that all of our family can be there.  By the next day, I was given a list of names of even more amazing “framily” (because really, people who rearrange schedules and take care of your crew are more than just friends) that are bringing meals the week of surgery.

Everything is coming together and we see God’s loving hand letting us know He is here and He is watching over us.

Here are some specifics to pray for:

  1.  For Emmie’s neurosurgeons and all the medical staff that are going to be involved.  This is not a common procedure on a child under two.
  2. Peace for all of us.  February 17th is going to be an extremely long day (and possibly night).
  3. For the kids to enjoy their time with their friends.

So, February 17th.  The battle with kyphosis and neurofibromatosis has been set.  We’re counting down the days and enjoying each moment we get.

12642717_10208537697463661_4119130811225715710_n

Facebooktwittermail

Christmas at CHOA

Emmie had an appointment at the beginning of December.  It was fun seeing the hospital all festive.IMG_20151204_104500518 Baby and Emmie were waiting patiently for an x-ray.
IMG_20151204_112651056 IMG_20151204_112722163 A small toy soldier army.
IMG_20151204_114554917 The weather was pretty decent and we had free time after the x-rays before Emmie’s appointment.  We wandered down to the koi pond.  On previous trips, they were doing construction and it was all closed.  I thought it was just a little pond.  Nope.  They have a small basketball area, green space to kick a soccer ball around and a small putting green.  IMG_20151204_114556643 IMG_20151204_114611796 Our kids had a wonderful time!IMG_20151204_114825387 Bryant had to get a picture by the turtle.IMG_20151204_114906360 Putting away!

I love that our kids are always up for an adventure and can have fun wherever they are.

Facebooktwittermail

Getting Real

Well, it’s been five months, an ER trip and multiple x-rays.  This is the magical time that we have been waiting on since Emmie fell in August. Originally, Emmie was supposed to go in on January 8th, but our neurosurgeon, Dr. C needed to see the CT scan first.  Fast forward a few weeks and then there we were.

Thursday Emmie had a CT scan under general anesthesia.  After she was done, we met up with Emmie’s neurologist, Dr. W.   Then Friday, we were back at CHOA to meet with Dr. C.

Emmie did wonderful on Thursday.  Naturally, she cried as soon as we entered her room in Day Surgery but she soon calmed down.  The nurses are starting to recognize us too.  1453383598194 This was after the “happy juice.”  That and whatever anesthesia they used made her think she was The Flash.  After she woke up, she tried to get up out of our arms.  When we were discharged and walking down the lobby to the cafeteria, she tried to run.  She didn’t run before the super meds…but yet she thought she could on unsteady legs.  We got a kick out of her trying to be so independent.IMG_20160121_065823067

We’re always a bit nervous on scan days because of the results we have gotten in the past.  This time, was not as painful.  From what Dr. W could tell, the plexiform (tumor) is staying in her neck.  Because she didn’t cry out in pain when we press on the plexiform, he also felt it was a good sign.  She smiled for him and allowed him to do a neurological exam.  She is still on track for being pretty much awesome.   We know the plexiform has grown from what we can feel but the specifics can not be seen except on an MRI.  Once the surgery is done and halo is off, she’ll be having an MRI to get a better look at the plexiform.

IMG_20160121_154412325

Little bit was super exhausted when we left CHOA.  When Emmie is anxious, she wants to hold our hands especially when she sleeps.

Friday was very hard.  We knew that the detailed appointment was coming.  We just weren’t prepared for the reality to settle in with all the details.  Now it’s real.  Now it’s game time.  We were reminded once again that Emmie’s spine at this age is not the norm for the medical community.  We are so grateful that Dr. C has been reaching out to other neurosurgeons across the country to make sure he is doing the best thing for her.

Before I dive into surgery details, I wanted to mention something.  Several people have assumed that the halo is the fix.  It’s not.  That’s easy to think because most of the time when an adult or older child has a halo, it is because they have a break.  Just always keep in mind that Emmie is different.  The halo brace was placed to keep her spine stable until her bones were strong enough to take a surgery to them.  The doctors did not want to keep her in a halo much more than five months without trying to correct the spine (halos fail after a certain amount of time and constantly have to be monitored.  Also, she has grown quite a bit since August).

cropped

This was the curve in August using MRI (the details on the spine are not as clear as a CT) and before the halo traction.

Emmie's Spine 1-21-16 cropped

This is the CT scan from Thursday, the 21st.

There has been some improvement from the scan in August but it has stayed completely the same since after traction.  The good news is that the halo has and is continuing to keep her spine stable.

She’ll have surgery sometime in February, probably toward the middle of the month.  She’ll be in the hospital for 7 to 10 days.  However, Dr. C estimated 3 to 4 days for the plexiform re-section in April and she was only there 24 hours.  We know we’ll be there longer than 24 hours but would be super excited if it was less than 10 days.   After surgery, she’ll remain in the halo for at least six weeks to continue to allow her spine to strengthen.  Then, the halo will come off and they’ll monitor her really closely for awhile.  If there is any indication that the spine is beginning to bend again, she’ll be put back in the halo.

Surgery is going to be long.  A lot of decisions they make will be when they start the procedure.  Right now the surgery consists of three parts:

  1.  They’ll remove more of the plexiform to get to the spine.
  2. Anterior-They’ll go in to the front and remove and replace two of the vertebrae (the two that are shaped like triangles but supposed to be rectangles). The replacements will have to be custom made for Emmie because they don’t make them small enough for her.
  3. Posterior-To provide more stability, they’ll put in rods in the back of her neck and will fasten those with either pins or wires depending on the bone thickness.

There it is.  The condensed version of what is going to go down in the coming months.

Dr. C thinks the world of Emmie and we are trusting God to provide healing for our amazing super hero.  Right now, I don’t know what to think.  My brain is in planning mode, but experience tells me to enjoy each moment with our family right now.  Now is when we’re reminded that we aren’t in control and that each moment is precious.

We’ve put together some shirts to go along with Emmie’s fight.  This campaign closes on February 1st.  Check them out here.

Facebooktwittermail

Updates

Our life looks a bit schizophrenic, I supposed.  One minute we are in full “normal” school, church, activity mode.  The next we have NF things.  Then we turn around to work on adoption things.  It’s pretty busy around here and we are getting good at switching gears on a dime.

Alas, I realize I have yet to update the blog on on all the NF/Adoption goings on.  So, here it goes:

Adoption Update:

In less than a week, Mark and I will drop kids and head to the airport to fly over the “big pond” and arrive in a new and very exciting adventure.  Our first trip to Y the Brave’s birth country is coming up. We won’t be bringing him home (as much as we would love to) just yet.  This appointment is for our official referral.  All total, we’ll spend about a week in country.

The Lord has provided for our funds for this trip with a grant and several donations and all of our hardwork (and friends and family too) with fundraisers.  We’ll have two more trips coming up and total need around $8000 to finish out the adoption overseas.  That will help pay for our travel, stay and other expenses in country (Y the Brave’s new paperwork, etc).  Right now, we were blessed with a covenant loan (no-interest loan) through LifeSong for Orphans for some of that remaining $8000.  We would love to not have to use any of that loan and are praying the Lord provides just as He has throughout this whole process.

My heart ya’ll, I can not wait and don’t think I will ever forget the look on Y’s face when we walk in to his school.  I don’t know how in the world I will keep from grabbing him up and smooching him (aside from the fact that it would totally embarrass him in front of everyone…I guess I will restrain myself).

Halo/NF

IMG_20150922_162519353

Our ER trip. Emmie fell and dislodged two of the pins in the front. Thankfully, Dr. C was able to hand tighten them in the ER (with some heavy duty pain meds to calm Emmie).

Emmie has had quite an adventure.  In the past three months, we have been to the ER once, pediatrician a couple of times, fallen numerous times, seen the orthotist a few times and now had a complete halo/pin site re-positioning.

IMG_20150922_185007782

ER trip under our belts and refueling for home.

IMG_20151007_124350406_HDR

This was after a super painful pin tightening visit and a dose of tylenol. Her strength amazes me.

 

She went in Tuesday and had the re-poisitioning done.  Her falls have caused her halo to get wonky and the pins to push in too far (not in to her skull but still too far).  Her x-ray on Friday, the 30th, showed some other issues too.  The pins were irritating the skin a bit and he wanted to put them in new sites to let her skin heal.  Dr. C knows we are in the process of adopting and wanted to get the procedure done before we left town.  We were glad he did.  She has been getting increasingly upset and agitated with pin cleanings and just in general.  When she reaches up to her head or the back of her neck and starts crying, we know she is having problems with it.

Dr. C, neurosurgeon extraordinaire decided that she needed to be put to sleep to get everything centered and set again.  Dr. C, unfortunately, was not going to be in town this week, so Dr. R., yet another amazing neurosurgeon and the one who originally placed the halo, did the procedure along with Super R the Orthotist.

IMG_20151103_094932529

Power nap before the morning activities at the hospital.

She did wonderful.  Even though she had to wait an extra two hours because of an emergency, Emmie experienced no problems.  We were able to go home in the evening and every one has been glad to be here.  So far, over-the-counter pain meds are working too!  She’s a tough one and we are so proud of her and how well she is doing.   IMG_20151103_102243998

This was a daddy trip.  She was only happy in Daddy’s arms.IMG_20151103_105502929 Just got the versed.  That is a magical medicine.  Unfortunately, the two hour wait meant that it had worn off somewhat by the time she went back and she was quite distraught at having to leave us.  That was the hardest OR release we have had yet.IMG_20151103_105843846_HDR 11216256_918140338279448_2600174791621421454_nAll done.  Yes, she does look taller.  Her neck was re-positioned and gained her a bit more inches.IMG_20151104_103359237

And the day after.  I got her all decorated up and she waited patiently while I did it too!

So, now we have our first OR outpatient done and third surgery.  She really is an amazing little girl.  I would be curled up in bed for a week after this one surgery but no, she is ready to go and explore.  And let’s not discuss the pin tightening that requires excess amounts of chocolate on my part…and after a good cry, she is ready to go!

Since we are three months in to halo land, we have about two more months now until surgery.  I’m guessing our next visit with Dr. C in December, we’ll start really discussing what is going to happen and the whens.

Facebooktwittermail

Brace Yourselves

One of my biggest shockers since Emmie’s diagnosis has been that I have been brave enough to drive through Atlanta without another adult, not once, but three times in the past three months.  While I don’t mind traffic, Atlanta traffic is its own little world that I didn’t care to delve in to.  Until Emmie required us too.

In the middle of June, we got the call that Emmie’s new brace (the one just before the halo) was ready and Mark quickly rearranged his schedule and took off so that I could take Emmie for the short appointment.  We both knew it would be a boring appointment for the kids but hanging out with daddy for a whole day would be totally awesome.  It was.

Since this was my first, “Emmie and me” trip, I made sure we had a celebratory pit stop.  At Ikea.  Oh yes we did.  There are some perks to having to drive to Atlanta for all of her awesomeness.

IMG_20150715_114736062The appointment took a bit over an hour and she did a wonderful job.  Despite the fact she was terrified every time she heard a door open or close.
IMG_20150716_072437_01

I must say, she works this brace like the princess super hero that she is.IMG_20150717_193617_261 IMG_20150717_193645_341

Complete with double pony tails.  Oh yeah.

I found an etsy site called Higgy Bears after Emmie got her brace.  This wonderful lady creates stuffed animal braces for children to have stuffed animals sporting their same sense of fashion.  I knew she had the scoliosis braces but wasn’t sure if she could make something similar to Emmie’s brace.  I decided it was worth a shot and sent an email.  And she did it!  I was totally impressed.  I haven’t had time to email to see if she could do a halo brace now.  But for now.  Her LeighLeigh doggy is a favorite.

IMG_20150805_165049147 IMG_20150805_165106934

She even went back for her halo placement procedure.  LeighLeigh, by the way, is after Emmie’s amazing orthotist who came up with this brace that was indestructible (stoppable only by the evil Kyphosis villain).

Facebooktwittermail

What now?

I’m sure everyone is wondering what the plan is now.  Our immediate plan includes five months in the halo or until Emmie is between 20 and 21 months.  She’ll then have surgery to place what Dr. C calls a “straw’ between either two or four vertebrae in the front of her spine.  They may or may not fuse the spine in the back for added stability.  They will go in to the front to do the work because that is where the bend is.

This is all up in the air but all the neurosurgeons agree that they do not want her in a halo past five months before she has surgery.  Once surgery is done, she’ll stay in a halo for about three months before they remove it.  Then three months after that, if she is stable, we are done with this part of NF.

Yep.  I said this part.  Remember, I said this was a marathon.  Unfortunately, NF is a progressive disorder.  But it is also unpredictable.  It is our understanding that we’ll be addressing the issue of plexiform growth after we get the spine situated and stable.  Of course, since all cases are unique and different, those plans may change too.

All we can do for now is enjoy our “new normal” and hang on and pray.  We may not know what in the world is coming at us next, but we do take great comfort that God knows it all and knew it all before we ever started this journey.

Facebooktwittermail