Back to Boston...

I know it has been another really long time since my last post.  Writing about Harlie is harder than it used to be.  Writing about her and our life with her was, for many years, very therapeutic. I found early on in her life that if I was going to share the negative, I had to find some positive to tell you about.  I had to show everyone that we were still hopeful, happy and grateful.  So, even during some really rough times, when I sat down to write, I found something positive to share.  And looking back, that wasn't really that hard - because I really was so hopeful that easier times were to come.

But, then August of 2017 happened.  Boston happened.  And that emergency trach took all of my hope away.  All of it.  It made finding the positive feel almost impossible.  So, over time, writing became to feel more of a burden.  Especially once we got home from her two month, scary, horrible hospitalization in DC (February - April 2018).  I often have to fight to keep myself from feeling angry.  We just lost so much by going to back to the trach life.  And the trach life has been so much harder this time around.  Insult to injury.  Salt in a wound.  So much more pain all around this time.  As Harlie's mom, I am expected to handle stuff that makes other people cringe and look away.  But, I can't do that.  I have to face it - and do so with a smile.

I have to hide my true feelings most of the time.  I have to hide my anger, my grief, my fears, my utter disappointment and defeat (which I am feeling all of the time) from most of my friends, my family and most of all - from Harlie. No one wants to see that.  No one wants to hear it.  Most people don't want to even think about it.  And, when it comes to Harlie, she needs me to be positive, upbeat, happy, grateful and funny.  That's what I want to teach her more than anything - to be funny.  😊

So, burying my feelings most of the time takes a lot of energy.  And I've been lacking in the energy department for quite some time now.  Most nights my sleep is interrupted by Harlie's equipment -equipment we didn't have to have when she wasn't trached.  More salt in the wound.

Anyway, in our never ending quest to give Harlie a better life, we had no choice but to revisit the craniofacial team in Boston.  This was done with some reservations:

1. I didn't want to go back. I didn't want to feel what I knew I would feel returning to Boston Children's Hospital.  Hello, PTSD!

2. Traveling with Harlie takes energy.  I don't have a ton of energy right now.

3. I was afraid they would say there was nothing they could do to help her.  Or that they wouldn't even try given the amount of close calls and challenges during all of her post ops.

But, what are we supposed to do - give up?  I don't want her to live the rest of her life this way!  And I've made a point in not letting fear make my decisions for me.  Plus, we did all we could to help her airway, and now there's nowhere else to go - but Boston.  So, I wrote her surgeon and told her what has happened, and what I was thinking.

Our appointment was Friday, July 12.

Thursday night was difficult.  My stomach was in knots.  While my mind knew it was just an appointment, my body was feeling the stress anyway.  So weird how that happens.  We don't have as much control of our feelings as people think we do.

We flew up to Boston Friday morning.  Got there and took an Uber to our hotel.  It was only about 10am, so we couldn't check in.  But, they held our bags for us so we didn't have to lug them around all day.  Then we walked to one of our favorite restaurants, Sweet Cheeks, for a quick lunch.  Then we walked to the hospital for our first appointment - her CT scan.

She held still for three minutes and all went well.

I want to go back for a minute to tell another story.  Back in August of 2017, the night of her emergency trach, I met with the anesthesiologist.  She was really nice, very good at communicating with me and told me about what happened in the OR that night.  But, it was so late.  It was well after midnight, and we were all so tired.  It was such a hard night all around.

Weeks later, while Harlie was still in the hospital there, that anesthesiologist came back to visit us.  I can count on one hand how many times an anesthesiologist has come back to visit after surgery.  She really wanted me to know how hard they tried to not trach her.  She wanted me to know they didn't make that decision lightly.  They had no choice - she would have died if they hadn't.  She spent some time with us that afternoon and it was great.  Here are some pics we took that day.

She gave me her card and asked me to send her those photos.  She told me that she would never forget Harlie or that night, and I believed her.

But, when we got home in September of 2017, life was so hard.  And I had nothing positive to say to Dr. Vinson.  I kept waiting for things to get better so I could tell her things were better and that we were okay.  I wrote her a card and wanted to mail it - but never did.  This is a perfect example of how the simple things can be too hard for a special needs mom.

So, finally, a week before our appointment, I emailed Dr. Vinson. I told her I was sorry for taking so long to share those pics.  I said I would love to see her when we were at the hospital if she was around.  But, if not, I wanted to drop something off for her.  She wrote back and said she wanted to see us, too.  She remembered, just like she said she would.  She gave me her cell number and asked me to text her when we arrived.

I packed that card I wrote and a We Heart Harlie & Friends hoodie.  But, as things often go, we freaking left it in our suitcase - at the hotel!  Ugh!  Simple things being hard.  I realized it at lunch.  So, I asked Tom to go back to the hotel and get it while I took Harlie to the hospital and her appointment.

And then Dr. Vinson found us in the CT Scan room.  So, I told her Tom was getting her gift and would be there soon.  She got called away and said she would find us later.

Then we went to dentistry to get images done there.  I sent Tom a text to tell him where we were.  Boston Children's hospital can be very confusing.  There are lots of separate buildings (we had three appointments in three separate parts of the hospital) to get to dentistry, you have to take one set of elevators to a floor, walk through that level, to go to an older part of the hospital, then take another elevator to dentistry.  I told him I was soooo tired and could he please stop and bring me an ice coffee.  He found us and I was grateful for the coffee.

Harlie was so cooperative for the dental images!  I know she was uncomfortable and her mouth/gums started to bleed during it - but she still did everything asked of her - with no complaint.

Then we headed over to craniofacial. After getting there, I realized I LEFT MY COFFEE IN DENTISTRY, which as I mentioned, was way too far away to go back and get.  The struggle is real, people.

I forgot to mention that on Thursday I got a call from a nurse practitioner from the craniofacial department.  She had some questions about Harlie.  She told me she was creating a PowerPoint presentation for the team so they would be prepared for us when we got there.  So crazy - my kid needs a freaking PowerPoint presentation!

Anyway, we went into the same conference room we have been in every time.  The team was waiting and Harlie's images were on the big screens on the wall.

Cutting to the chase, they confirmed what I have believed the whole time.  The jaw fusion release (ankylosis release) on August 8, 2017, set her jaw back, into her airway, causing an upper airway obstruction.  I remember meeting with one of the surgeons immediately after her surgery.  He said he had to remove a centimeter of bone.  I was shocked, a centimeter is a lot when we worked so hard a few years ago for MILLIMETERS.  So, I asked, "Is that going to negatively affect her airway?"  No, he said.

But, doctors are people, too.  They are not right all the time.  And each patient is different.  Especially Harlie.  But, I knew.  And I need to remember that. I need to have confidence in my gut and what I know, even without a medical degree.  No one knows Harlie better than I do. 

While there are several options out there, in her case, there is only one real, viable option - mandibular distraction.  That is what she had in July of 2013.

Here are some images from her CT scan.  Air is black and there should be black going all the way from her nose and mouth, down her throat and into her lungs, with no interruption.  As you can see, she does not have an open airway.  This is her profile.  Also, notice her cervical spine defects.

The surgeon said that it is likely that the back of her tongue base is touching the back of her throat at times.  Ugh. Can you imagine how annoying that must feel for her? No wonder she coughs all the time! My poor girl!

Mandibular distraction is when they break the bone, put pins and rods on both sides of the break and everyday we turn the screws and it re-breaks the bone - spreading the break apart, allowing new bone to grow in the middle.  This will (hopefully) make her jaw longer, so her tongue base can have room in her mouth and get out of her airway.

However, it isn't quite that simple.  After the last distraction (which was successful) her jaw bone kept growing - but towards her ear (which is not good) and it kept growing until it touched the base of her skull.  That is how it fused closed.  And that is why she had to have the release in August 2017.

So, she basically has no jaw joint.  Which means that when we go to distract her jaw bone again, it would spread apart in both directions.  We only want it to grow in one direction - towards her chin, not towards her skull (this would certainly cause the fusion again).

So, they are going to install some hardware to give the jaw stability - so it only grows in one direction.  They pulled up another patient's CT scan so we could see an example of the hardware they will use on Harlie.  Here it is:

The rods that go in above her ears will be exterior and are there for stability.  Nice, right?

We will turn screws for roughly three weeks and she will have to wear the hardware for two to three months, I think.  Those time tables could certainly change, depending on her bone cooperation and growth, of course.

After they went over everything they asked us how we felt about doing it.  Here is my thinking:

1.  The risks we have to consider when Harlie has surgery are the same - no matter what surgery she is having.  The risks are not greater with this surgery.

2.  She does not have a good airway.  If her trach were to come out and we couldn't get another one in, she would not be able to breathe.  This is a hard fact to live with, and quite frankly, I find this riskier to live with than any surgery.

3.  The scar tissue around her trach is horrible.  It flares up (bubbles up like a blister) and bursts on a regular basis.  This skin can only handle so much trauma.  And it is painful for Harlie.  There is no resolution in sight.  Other than getting that trach out and letting that skin heal for good.

4.  Her quality of life.  She knows what she's missing.  She sees what other kids can do and what she can't.  I took her to the pool a while ago and I had to hold her on my hip and walk around like she was a baby - she will be 13 next month.  It was raft day at the pool, and she had to watch all the other kids play freely on rafts.  Sometimes I don't know how she deals with her sadness.

While hearing all this was initially really difficult, I quickly made the transition to relief.  I was so grateful to hear that they think they can help her.  I was really afraid that we were out of options.

After we told the team we want to proceed, Dr. Padwa said, "Okay, team, what do we need to do to entertain operating on this kiddo again?"  Haha!  So, they started to brainstorm who needs to be on board - ENT, cardiology, etc.  She said she wants Harlie to come up one month before the surgery to see all her specialists so they are all comfortable with a plan.  Then we will go for the surgery, come home for the turning and recovery.  Then go back up again to have the hardware removed.  That's a lot of travel.

Oh, and while we were still in the conference room, I received a text from Dr. Vinson.  She said she had to leave the hospital soon but wanted to see us first.  So, I texted back that she could just come to us then.  It was so funny because right about then we started joking with her docs.  Dr. Padwa was talking about that night (August 10, 2017) and was telling the team how she was in bed when she got the call from the hospital that Harlie was really struggling to breathe and needed to be reintubated in the OR.  She told them she threw on her scrubs and rushed in.  She said she was freaking out wondering why it was taking so long to get her down to the OR.  I do remember her yelling, "WHAT IS TAKING SO LONG?!"  It was absolutely awful to watch Harlie struggling to breathe.  It makes my stomach hurt when I remember sitting by her bed that day.  We all made her struggle for too long, hoping and praying she would turn the corner.

Anyway, Dr. Padwa said something like, "I was freaking out and here was mom, being calm, making jokes and asking ME if I was okay!"  I had to tell them that is just the way I cope.  I really don't like serious situations, so I try to make it better by joking and if you don't know me, you might find me inappropriate.  I am pretty calm in those situations (sad that I can say "situations" and that I know how I behave in them).  I want the team to be able to focus solely on Harlie and not have to worry about me.  I probably also don't want to show how freaking scared I am.  Because trust me when I say those situations are super fucking scary.

Back to the story, Dr. Padwa said, "I remember the anesthesiologist that night."  And then there was a knock at the door and it was Dr. Vinson.  Haha!  Dr. Padwa was so surprised!  She said, "Amy, I was JUST talking about you!"

So, Dr. Vinson joined us and we finished up our consultation.  So, we are waiting on a surgery date now.  My guess is we will have to wait until spring 2020.  We'll see.

Okay, I'm going to wrap this one up.  This was long and took forever to write.  I guess it is appropriate that this one gets done close to the two year anniversary of that stuff.  I have a lot more feelings about all that - might share them in another post.  We'll see.  At any rate, this week is a tough one for Tom and I, with lots of big, high and low memories.  Thanks for thinking of us and cutting us some slack. 

Much love,
Christy xo