I’ve been trying for two days to come up with what to say here.  An update for Emmie.  Something I definitely don’t want to type.  But, I know that we have so many friends from all over who are praying for her and us that I feel I need to update you all too.  I just really have no idea how to put a title on this one.

Thursday morning we received a call from the neurosurgeon’s nurse practitioner.  She told us that Dr. C had consulted around and wanted to talk to us about surgery.  Obviously, if a doctor wants to meet with you, it’s not good.  We’ve learned that pretty quickly.  However, because Dr. C is two hours away Mark set up to talk over the phone.  We were supposed to talk Thursday afternoon but after not hearing from him at the appointed time, Mark called the office and they told us he was in surgery.

So, we waited.

This is the interesting part.

I was out getting Easter clothes for the boys while Mark was cutting grass.  One off the kids was in charge of watching Emmie.  And that child was watching her.  However, the child had set her on the couch, while the child was watching her…she fell off.  Thankfully, the child caught her before she fell all the way on the floor but she led with her hands.  I got home ten minutes after the episode and she was still crying and not using her hands too much.

A quick call to the pediatrician and I whisked her up and got her there faster than I will admit.  Dr. B sent her on to x-rays next door and called the radiologist to let him know about her NF.  They also gave her a bit of motrin.  Quick x-rays and we were back to Dr. B to await results.  Thankfully, by that time, she was finally settling down and with her arm braced against me, went to sleep.  Over an hour of pitiful painful crying.  I was heartbroken and Dr. B was getting worried.

Thankfully, the x-rays showed nothing was broken.  The best that Mark and I can figure is that she sprained her wrists.  She continued to favor her wrists the rest of the night but did use them some.  Saturday was a bit better.

So, on the way home we got the call from Dr.  C.  He had consulted with ENTs, general surgeons, and everyone else in between all across the country.  They were all in consensus, something needs to be done sooner rather than later.  The reasoning is that the worse the kyphosis gets, the harder it will be to repair it and the less successful it will be.  Dr. C looked at other options besides surgery but Dr. W confirmed that chemo would not be good at her age or for the type of plexiform neurofibroma.

That leaves us with one option…surgery.  A general surgeon and Dr. C will both be working on Emmie.  We should be hearing from the scheduler within the week with possible dates.  We do know that it will be within this next month.  Because the kyphosis hasn’t gotten significantly worse in the past six months, we don’t want it to keep heading in that direction.

The hope is that the surgery, which will remove as much of the plexiform as possible (called debulking), will significantly correct the kyphosis.  Again, the goal is to try and delay the kyphosis correction as long as possible because her bones are so soft (due to age).

I feel like I’m typing somebody else’s life right now. Not ours.  But here it is.  If you ask how we’re doing, we’ll be honest…not good.  We know God is in control.  We definitely trust Him.  But this is so hard.

We will update when surgery day is scheduled.  For now, if you feel led to pray, here is what God has laid on our hearts:

1.  For steady hands and wise doctors.
2.  I am feverishly trying to plan out the rest of the kids’ school so that they can work on some of it while we are in the hospital and recovering. Please pray I can get it all done and getting all the normal travel things ready.

3.  Peace for the kids and us and our family.
4.  Good coordination of schedules with grandparents, aunts, or other childcare volunteers.  We have expressed to both our sets of grandparents that we would like for them to sit with us during the procedure.  That means we’ll need childcare for the children who won’t be at the hospital.

When we had Emmie’s welcome party, we handed out little cards and told visitors that, as they felt led, they could write a verse or prayer for Emmie and our family.  No one knew what we pretty much suspected.  No one knew that those cards would be read over and over again and fill my heart with God’s peace over and over again.  When Emmie went through the initial diagnosis and first MRI, we received several emails and texts and messages with verses and prayers.  I wrote those out on the cards and added them to the ones we had received at her party.  This is where they are now:

She’s now sleeping in the girls’ room so these will be moved eventually but for now they hang over her crib underneath is a beautiful picture my grandmother had started sketching but had never finished.  As an aside, I remember her telling me that she didn’t like the way it was turning out so she stopped it.  To me, though, it’s beautiful.   So, when I rock and nurse Emmie and am praying over her I can look up and see God’s promises and friends’ prayers for our beautiful girl.

Now for the update.  On Friday, we traveled early in the morning to Atlanta where we met Grandmere and Papa who sweetly volunteered to take the crew back to their house.  After bugging the neurosurgeon’s office we had a better feel for what the day held and knew it was going to be a day full of appointments and fittings.  Not something we wanted to make all eight kids go through.  Have I ever mentioned how amazing our family is?  We had scheduled Emmie’s appointment with the neurosurgeon for Friday on Tuesday.  Three days notice and our family made it happen.

After dropping the kids off, we made our way to Emmie’s appointment with the neurosurgeon.  Dr. C was great at explaining and showing us Emmie’s kyphosis.  We found out that there are probably two things that are causing the abnormal bend in her spine.  First, the weight of her head pushing down on the spine.  Secondly, a plexiform neurofibroma (tumor) pushing in to the spine causing the curve.  From what we understood, the plexiform was not noticeable in September but was definitely visible now.  Honestly, we had no clue about the plexiform being there.  My heart just dropped when he told us.  Just not what I was expecting.

This is as if looking at her profile.  The curve towards the top of the spine is the kyphosis with the tumor being in that curve closest to her chin.

Here’s the plan:
1.  Dr. C is looking for an ENT who has experience with a similar situation.  He wants to find out if it would improve the kyphosis to debulk the plexiform now.  Please pray that Dr. C will find the right ENT. Words used were “complex procedure” and ” difficult to find someone who has dealt with this.”  Two things that took our breath away.
2.  Emmie will wear a cervical collar.  The collar will hopefully take the pressure off her spine from her head weight and keep the kyphosis from getting worse.
3.  The goal is to delay surgery to correct the kyphosis as long as possible.  The surgery will involve rods and pins in the bone (I can separate myself now from the medical and the emotional when I’m typing but please know that I am not typing this halfheartedly…I know it’s a lot to take in when we are talking about a baby).  Obviously, babies have very soft bones so the longer we wait the better chance she has at it being a successful surgery.  As long as she is not experiencing any neurological symptoms or problems, surgery is not necessary.
4.  To monitor the kyphosis and get a more accurate measurement, she’ll have an x-ray every three months starting this Friday.  We have learned that x-rays show bones better than MRIs just like MRIs show soft tissue and tumors better than x-rays.   Getting x-rays and being exposed to radiation when we know that some people believe that the radiation exposure causes the tumors to grow is not ideal nor what we would choose.  But we know it’s what is needed for now.

After all of that news, we headed across the street to the hospital to get the x-ray and then to orthotics for the brace fitting.  First, we had to refuel and get Emmie a quick nap.

The x-ray went quickly.  Just two quick pictures and done.

Orthotics didn’t take long either.

But I do have to say that putting that brace on my daughter while she cried was the hardest thing I’ve ever had to do as a mom.  Ever.  And I get to be the lucky one to do it.  She’ll wear it while she is awake and she doesn’t wake up in the morning until Mark is off to work.  Thankfully, I’m stubborn and while my heart breaks to hear her cry when I put it on, I know that this is what she needs now.

The difference in her head posture with the brace on was instantly visible to us.  And, again, made me catch my breath.  We just didn’t realize how much her head bent down.  She’s our warrior.  Our true hero.  After a day and a half of wearing the brace, she has already figured out how to turn her body to turn her head. She’s still cruising and playing and laughing and squealing like before.

Not only is she acting like our Super Emmie, she’s also getting more steady on her feet.  Friday night, she let go of my hand and briefly stood on her own before taking two very tiny steps to me.  She’s trying to pull up on more things too.

I know there are people far and wide praying for our super hero.  We are so grateful for that.  Here are a few things that are on our heart to specifically pray for.
1.  As mentioned already, that Dr. C will find an ENT to consult.
2.  Wisdom for the doctors as they decide the next step.
3.  Wisdom for us as we seek to make sure she is not having any issues from the neurofibromas or the kyphosis.
4.  Strength for our kids.  My heart broke as we arrived back at Papa and Grandmere’s house and Malachi watched me put Emmie’s brace on (she had taken the velcro off in the car right before we got to their house…stinker).  He was so worried and upset.  He recovered quickly once he realized she was okay and not hurting but still…I’ll never forget the look on his face.  Then Saturday, Malachi asked Mark, “What dat thing on her neck for?”  Mark explained it again and he said, “Oh.  I need a hug.”  And gave Mark a big ol’ bear hug.  It’s no secret that Emmie has seven guardians who will do anything and everything for her.  They love her passionately and will protect her fiercely.  This is a new thing and a visible thing that will remind us daily that we are running an NF marathon with her.
5.  Also, yes, we are used to comments and stares but her brace are going to cause more and more of them.  Please pray for courage and wisdom for our children as they share our journey, bring awareness, and hopefully, shine Christ’s light with curious strangers.  We’ve already told them that if they feel the questions are too hard or too much to just bring the questioner to us and we will help.

If you feel the Lord leading you to share prayers or Scripture for Emmie, just email or text me and I will definitely add it to her collection.  One day, I pray she will find comfort in the words as we have.

Now, presenting the latest in spring babywear (and on a beautiful model, I might add):

Ceili Rain said that the brace makes Emmie even cuter.  I decided that was because it made her full cheeks fatter.  Instant fat cheek lifter.  That means that her adorableness is even harder to resist.  Adorableness is her new super power.  Go ahead, I dare you not to smile.  The tears in the eye were from a long tiring day.  The whole walk back to the car she was talking and laughing and squealing.  So, she was fine….just completely exhausted.

So, our WAW is turning in to a bit more proactive.  Our pediatrician called last Monday and let us know the results of the MRI.  I should say our pediatrician called at 6:30 Monday night.  As soon as she received the MRI results.  We had looked at the scans beforehand and I had sent an email to Dr. B to let her know we were very interested in receiving the results as soon as possible.  She willingly obliged.  She not only went over the report but also calmed some fears and discuss some things to watch for.  We are blessed.  Beyond blessed to have a pediatrician who knows her stuff and knows NF!

Dr. W called later in the week  after we pestered him and he pretty much confirmed what Dr. B said.  He also confirmed that we are moving from an “if” she has surgery to a “when” she has surgery.  He was waiting to get information from Dr. C, her neurosurgeon before contacting us.  And, in true amazing Emmie fashion, she “won” a spot in joint conference, where all the neuro people discuss the more diffficult cases.  Go Emmie.

Here’s the results:

While the plexiform neurofibroma (benign tumor) that is on her neck is growing and pushing on things it is not causing any problems as of right now.  That is good news but is going to require closer monitoring.  We have been armed with what to look for if it starts causing issues and ya’ll know my mama hawk eyes are on it.

The main area of concern right now is the cervical kyphosis.  Kyphosis is an abnormal bend in the spinal cord (ya’ll know this is the non-medical me talking here right?).  In Emmie’s case, the spine is curving in the neck area.  It was noted on her MRI in September and we were told then that it was a WAW situation.  It could have been because of the way they positioned her in the MRI.  This time, however, it was a lot worse and very clear that it is not a positioning issue.  Her spine is now curving into a “C” shape with the opening being at the front of the neck.  Googling a normal spine MRI and you will see that is no where near what it actually is supposed to look like.  They don’t believe that this is directly related to the plexiform neurofibroma but it could be an indirect issue or just another one of those lovely NF symptoms.

Here’s a great article on orthopedic issues in NF1 patients.  Kyphosis is mentioned and treatment therapies are mentioned.  My social work nerdy self found it all very informative.

Surgery to correct the kyphosis will be in Emmie’s future, unless the Lord decides to heal her of it completely first.  However, the doctors want her to be as strong and as old as possible to ensure the greatest amount of success.  While, we all know that bracing will not cure the issue or completely correct it, they want to try it first to try and delay surgery.  So, at some point, Emmie will be sporting the latest in headgear fashion of some sort.  We don’t know how long in a day she’ll have to wear it or how long in months she’ll wear it.  Not sure what it will affect in her development or in her current muscle strength.  A lot of questions that will be answered at upcoming appointments.

We had hoped that we could go at least six month between MRIs but the plexiform growing and the kyphosis earned  her a three month vacation before the next one.  This will be MRI #4 in under a year.  I hate this part.

But this goofy girl just has my heart!

I know I’m not quite done with January updates but ya’ll, this girl’s pictures just had to be shared.

See?  She’s just adorable!

She had her nine month check up this month (March).

Here’s her stats:

18 lbs 8.4 oz.
28 1/2 inches long

She’s our petite little thing and we’re watching her weight closely but she is right on track with her development and we couldn’t be any more pleased!

She loves to eat whatever we have and expects all food to be shared.  She hasn’t learned that mama doesn’t share chocolate with anyone.

She’s not really interested in crawling but let her use you to stand up and she’s off cruising around whatever she can reach.