Surgery on Friday!

I can't believe it's been so long since I last blogged.  Time seems to be flying by.  We've been busy.    Somehow every day has filled up with stuff - and not necessarily fun stuff, either.  I really wanted to be able to relax a little and just enjoy some time with the kids.  After all, for us there is only a few more days of summer.  In fact, the boys leave to go and stay with Grandma (Tom's mom) on Wednesday!

We did manage to get in some fun... on Friday, me, Terri (our nurse) and Maggie (my niece) took the kids bowling.  Thanks for the great suggestion Bethany!

One thing I've really been struggling with this summer is handling germ exposure to Harlie.  Do I put her in a bubble and not let her do much or do I let her have fun?  It's something that goes through my mind constantly.

To get serious for a second (not usually something I like to do) after our experience with her cardiac arrest in the OR two years ago, I can't help but be fearful that something really bad could happen.  I've linked back to that post so many times.  I'm sick of hearing about it myself!  And I keep thinking, Geez! Get over it, Christy!  It was two years ago for crying out loud!

But, I suppose almost losing your child sticks with you for a while.  I can remember each episode that could have gone the other way (and yes, there's been several!), like it happened yesterday.  Especially when she's scheduled for a 10+ hour surgery.  That's a looooong time and a lot can happen in that time frame.  Not to mention the balancing act for the first 24 hours after coming out of the OR.  In the OR they will load her up with fluids and then the next day the challenge will be getting that fluid out of her.  Not to mention pain management (that's been scary in the past, too!). So, yes, I am terrified.  And can't help but think, What if the worst happens?  And I made her live in a bubble and didn't let her have any fun?  Those are some crummy thoughts to have stuck in your head.

So, I took them bowling.  They LOVED it.  The last time we went bowling was when Murphy turned five - it was his first birthday party out of the house.  That was three years ago!  Well, Harlie remembered exactly what to do.  She carried the six pound ball to the line, gently placed it on the ground and then pushed it with both hands down the alley.  All by herself.  And then she would turn around to walk back and smile proudly.  It was really cute.

Cooper would roll the ball down and then run to the ball return and hold his hands right there ready to catch it.  But the ball would still be rolling towards the pins.  I laughed every time.  He cracks me up.  Everyone had fun and I will have to remember to take them more often than once every three years.

So, yes, the big surgery is Friday.  This Friday.  Holy cow!  The past few days she has been pretty junky.  Last week when I took her to her ENT follow-up appointment we discussed that her secretions were really sticky.  So, her ENT said to increase her water flushes by a fair amount (like 10 ounces per day).  That would mean she should get 35 to 40 ounces of water per day, which seems like a lot to me.  She only weighs 37 pounds and I know for a fact that my boys don't drink that much water - or even close.  But, we did it.  And I really think it was too much for her.  I think she may be a little fluid sensitive.  My guess is her lungs just can't process that much fluid.  Her secretions were no longer sticky - but now they were more than plentiful!  Ugh.  I'm not sure which is worse!  For several nights we had to get up to suction her like 20 freaking times.  Which is pure torture in my opinion.  I would just lay down and fall asleep and I'd have to jump up and run in there to suction.  Then go back to bed, just fall asleep and it would happen again.  And again.  It was awful.  I increased her breathing treatments and the past few nights have been better.  But, I'm still holding my breath and hoping and praying that she doesn't get worse.

So the plan now is that Tom will drive the boys to meet his mom halfway to Pittsburgh on Wednesday.  They will stay with her from Wednesday to the next Friday.  They are going to visit their aunts and cousins who they don't get to see very often, so they will have a lot of fun. Then she will drive them home and stay with them here until we get back.

We leave on Thursday.  We bought one way plane tickets since we don't know when we'll be coming home.  Tom is busy working on the Ronald McDonald House renovation and he said if he can get that project to a certain stage before Thursday, then he should be able to stay in Boston the whole time.  I really, really hope that happens.  He has not taken off that many consecutive days off from work in years and years.  In fact, the last time we had a vacation I was pregnant with Cooper (that was the summer of 2008) and even then he would drive to work for several hours some of the days.

Rooney is going to stay with Brandy while we are gone.  Ugh.  This little trip is going to be tough on all of us for so many different reasons.  I'm worried about Cooper being gone for so long.  He's never done that before.  And this morning I went and did my Adrenaline class for an hour and when I got home he said, "Mommy!  I missed you!" and hugged me.  I'm thinking nine days might be a little rough on him.

So, this is what's going on this week:

We have a meeting tonight about a potential 5k run to benefit Harlie.  I will provide more details tomorrow.
I have a meeting today with the teacher that put together Harlie's "My Name is Harlie" book for school.  We are making another, up-to-date one this year.
I'm getting my hair cut tomorrow. Woohoo!
Then Harlie has a daisy girl scout pool party at our pool (the boys will go, too).
I'll pack the boys up and send them off Wednesday morning :-(
Harlie has a kindergarten screening appointment that morning with her new teacher (I'm very excited about our new set-up this year).
Then it's date night for Tom and I.  We're going to see the band Train with some friends.  I've been so looking forward to that!  It's kind of become a "tradition" for lack of a better word to go out with friends before a big surgery.  It's good to get our minds off it and to laugh and have fun with friends before the seriousness of surgery.
Then we pack up on Thursday and off we go.
Surgery is at 7:30am on Friday and we have to have her there at 6am.

We are staying at a hotel about three blocks from the hospital (not the same place we stayed last time, thank goodness!).  And that is courtesy of our wonderful, kind and generous neighbors - John and Jackie Hudson.  They are taking care of our hotel costs for the week.  We are very lucky people for sure!  Thank you so much John and Jackie!

And just to summarize what surgery this is (I've had a few people ask), here it is in brief:

It is called Fibula Free Flap Mandibular Reconstruction.  They are taking a good portion of her fibula (apparently an unnecessary bone in between your knee and ankle) and the vascular tissue around it and implanting it into her jaw on the right side.

She's already had two jaw reconstructions - but they used her cranial bone - and with no vascular tissue.  They were unsuccessful.

This is completely different and has a better chance of working because of the vascular tissue.  They are going to connect the blood vessels in the graft (what's taken from her leg) to the blood vessels in her jaw.  The hope is that the bone will have a nutrition source, and thus, will grow and heal.

They are also going to fix her left eye.

We are unsure if they are going to wire her jaw shut or not.  And we are unsure if she's going to be in a cast due to the harvest of her fibula.  Interesting note - when they take her fibula, they are not replacing it with anything.  So her bone will come down from her knee and just end.  And then her bone will come up from her ankle and just end.  And nothing will be in between.  Crazy, huh?

Okay, that's all I have time for now.  More later!  Thank you for caring about us!
~Christy xoxo

Boston and their Surgical Plan

For a quick trip, it sure felt long!

Harlie loved the plane ride.  Once she saw the tray table come down, she couldn't stop playing with it.  She sure wore that poor thing out!  Luckily no one was in the seat in front of her.  She tried to watch the tv and a movie on the iPad, but the headphones were not staying on her head.  And when we put the headphones over her hearing aid, it gave us feedback and buzzed.  We'll have to figure out something for her for next time.

And yes, there will be a next time.

After we got into Boston, we checked into our hotel.  We got an early check-in (we arrived at 9am) so we could put our luggage down and head on over across the street to the New England Aquarium.  We could see it from our hotel room.  A HUGE thank you to our wonderful neighbors, John and Jackie for providing the hotel (and a wonderful surprise dessert - chocolate covered strawberries, champagne, cookies and milk!).  They are so good to us!  We are so blessed to have so many wonderful people supporting us!

The aquarium was really nice.  But it was "vacation week" for the kids up there, so it was crowded!  After about an hour and a half, she was done.  I didn't think she had walked that much.  But, I think the crowd got to her.  And we did wake her up before 4:30am, so I guess that's to be expected.  We took her into the gift shop to see if she wanted anything.  She picked up a few things and examined them, but put them back.  Then she saw this fish...

went and grabbed it, and sat in her stroller and that was that.  No more looking - she was done.  We were very surprised considering it's a plush toy (not always been her thing).  But, she must be coming around. She didn't let go of it the whole time we were gone.

After the aquarium we went and had lunch.  Then we headed over to the Children's Hospital.  We got to meet a mom who I'm friends with on Facebook.  Her daughter has Goldenhar Syndrome, too.  She lives up there and she had her daughter and son with her.  They were there for something else and came to the craniofacial clinic to meet us.  So, that was really nice.  Thanks, Tanya!

After only a few minutes in the waiting room they called us back and took us straight into a conference room where there were about four doctors already sitting.  Already sitting!!!  Harlie's CT scan images were already up on two large flat screen tvs on the wall.  After introductions, more docs came into the room, until there were ten total.  Harlie was unfazed and found some tongue depressors to play with while we started discussing her future surgical options.

Overall, we were very pleased.  Dr. Bonnie Padwa spoke first.  She said, "You came here for a second opinion and that's what we're going to give you - you might not like everything you hear."

No prob, we can handle it.

The only thing that she said that was weird is that the term "Goldenhar Syndrome" is an outdated term.  They call it Hemifacial Microsomia.  But, in Harlie's case you'd have to add a whole bunch of other stuff to that.  Personally, I don't really care what it's called.  We're well aware of the issues and are addressing them, so whatever you call it, it doesn't really matter.  The only thing that Goldenar does vs. hemifacial microsomia, is that it's a hell of a lot easier to say when explaining it to family and friends.  Here are some things interesting things about it:


At the current time, no specific gene has been identified that causes this condition, and it is very rare for someone with hemifacial microsomia to pass the trait on to his or her children. The exact cause of this condition remains unknown but there is some experimental evidence published many years ago suggested that if a small blood vessel ruptures near the developing ear in mice, before they are born, after birth they appear to have hemifacial microsomia. More likely, this condition results from an impaired flow of cells (called neural crest cells), which arise next to the spinal cord, and migrate to the face to form the facial skeleton; if not enough of these cells are able to successfully migrate to their intended location, that side of the face ends up being smaller.


Children with hemifacial microsomia have a smaller face on the side that is affected. Parents usually note that the corner of the mouth is higher on this side and that the chin does not exactly line up in the middle of the face. The lower jaw (mandible) is flatter and shorter, and the ear is either smaller or not formed at all (microtia). The jaw joint (TMJ) may be small or even completely absent. Some children will have a cleft extending off the side of the mouth (called macrostomia), making the mouth opening larger. Some children will have weakness of the muscles on the affected side of the face. The child's right side is more likely to be affected than the left (2:1), and up to 25% of cases are bilateral (Bifacial Microsomia). Some children are very mildly affected, and others are significantly affected. Intelligence and development are normal.

If a child has a bump, or benign tumor of the eyeball, called an "epibulbar dermoid", then your child may have Goldenhar variant, which may be considered another type of hemifacial microsomia. Children with Goldenhar frequently have fusions of some of the vertebrae (spine) in the neck; however, this almost never requires any treatment.

Harlie's right side is definitely the more affected side in terms of her jaw.  However, it's her left ear that's missing.  I do think something is different about her mouth opening.  And it's something that we've discussed with her previous plastic surgeon.  He thought that her mouth was more open on the right, which you can see if you know what you're looking for.  But it's the muscles on her left side that are more weak.  When she smiles, she can't bring up the left side of her mouth like her right.  


And she does have a dermoid on her left eyeball.  I think she has one on the right, too, but it is covered up by the skin around her eye, since that eye closed properly.  You can only see the one on the left because it is exposed.  I'm sure that dermoid is bigger than the one on the right, which probably prevented her left eye from closing.  And she does have fusion in her cervical spine area.  


Anyway, Dr. Padwa went on to explain what they think would be the best option to get Harlie's jaw more functional.  They would do a fibula flap reconstruction.  They would take bone from her fibula (shave it from the side), along with vascular tissue around it, and then implant it into the right side of her face.  

Because they are transplanting the vascular tissue, and reattaching the blood vessels to vessels in her face, the bone will live and stay in place.  Her previous two jaw reconstructions were done just using bone (from her skull) with no vascular tissue, and of course, one of the grafts did not survive and had to be removed two months later (on the right side).  


Here are the positives:


1.  No craniotomy!!! The past two reconstructions were done by using bone from her skull, which meant they had to cut her head from ear to ear.  That was awful.  For many reasons.  And definitely made the recovery much more difficult.  It also made for more swelling and bruising of her face.  Plus, it very negatively affected her hair style!  So, all that would not be an issue this time around.  Woohoo!!!!


2.  No wires!!  If her jaw is wired for recovery, it would only be wired for a few days and she would not come home with them.  So, no jaw wired shut!  For 9 weeks like the previous two reconstructions!  Woohoo!!!


3.  No cutting of her jaw!!  They would take the bone from her fibula and put it over what's already there.  First, Dr. Padwa would put the jaw in the desired position, so her teeth line up, and then they would secure the bone in place with plates and screws.  All of this means for less swelling and bruising and no black eyes swollen shut.  Woohoo!  


4.  Her hospital stay would depend on how she does, of course.  But, they think it would be about 7 to 10 days.  


5.  They said they could fix her left eye at the same time.  This would mean an additional two surgeons, but they said they can arrange it.  I wonder what fixing her eye will do to her appearance.  I think that's one thing that kids notice first and I believe they find it unsettling.  Let's face it, the eyeball is kinda gross.  So, seeing more of it is a little weird.  It's both exciting, and scary, to think of what she's going to look like after all this work.  I don't think I'll ever get used to her face having to change so quickly due to plastic surgery.  It's quite difficult to experience, and explain.    


Here are the risks:


1.  Ankylosis - the stiffening or immobility of a joint resulting from disease, trauma, surgery or bone fusion.  They are 100% confident that the bone will take and will live after surgery.  Sometimes it takes too well and overtakes the joint of the jaw.  They will measure the opening ability of her mouth every day and see if the number decreases.  If it does, he'll have to go back in and adjust things.  I should probably know more details than "adjust things", but I don't.  The bottom line is that the risk of ankylosis cannot prevent us from doing this surgery.  So, we'll just have to cross our fingers that it doesn't happen, and if it does, deal with it then. 


2.  Facial nerves.  Since her face didn't form normally, one cannot assume that everything is where it normally is under the skin.  So there is a risk of hurting facial nerves causing paralysis.  I believe he said that he will be going under the nerves and that most of the time the damage to the nerves is temporary and in time, they get better.   


3.  The normal risks that are present anytime one (especially Harlie) undergoes surgery.  This has no impact on our decision whatsoever, as we cannot do anything about it.  This is not a voluntary surgery and it is in no way cosmetic.  It is to gain function and a better quality of life.  Therefore, we must proceed forward.  And it doesn't hurt that Children's Hospital Boston is listed as the number 1 ranked children's hospital for cardiology and heart surgery.  So, our biggest worry gets some comfort.


So, as you can see, the decision is a relatively easy one.  Notice that I said risks vs. negatives.  There really are no negatives to the surgical plan that we can see at this time.  But there are some negatives to choosing Boston for surgery:


1.  Distance.  It is 550 miles away (about a 10-hour drive).  We flew Jet Blue and it took us one hour to get there, and one and a half hours to get back (headwind).  It's guessed that we will have to go up there at least five times, bare minimum, relating to this surgery - if all goes well.  And that's also assuming that we can get a lot of the tests needed prior to surgery, here locally and have them sent up.  


2.  Money.  It is out of our network for insurance, which changes things drastically.  And they are not enrolled in Virginia Medicaid.  They are only enrolled in neighboring state's programs.  After paying the deductible, our insurance will pay 70% and we pay 30%.  But then the hospital has the right to balance bill us, which means whatever insurance did not pay the hospitals/doctors, they could bill us for that amount.  Pretty scary when you think that this will total in the hundreds of thousands when it is all said and done.  Clearly, we will have to study our insurance plan more closely.  And this week I will call the hospital and ask them about any plans or programs they have for people in our position.  People come from all over, so we are certainly not the first to be in this position.  So, we'll see.


This quick trip was super expensive, but we learned a lot, so we'll be able to be smarter next time.  I hope.  


After talking in the conference room with all the docs, Dr. Labow showed up at the end of the meeting.  He is the surgeon that would take the bone from her leg and place it in her face.  Since we had more details to go over, we met with him separately and spent some time going into more detail.  


He looked at her leg and said she had enough leg to be able to get what he needed and she had a great pulse, so that means her vascular tissue is good and strong.  


He examined the right side of her face closely.  After that he asked me about her history with vascular access from her neck.  I told him that they have had to gain access through her jugular numerous times before because her femoral veins (in her legs) are shot/scarred (from so many heart caths and surgeries).  I told him that I thought it had always been done on her right side (if memory serves) because she has two... (and I snapped my fingers as I was trying desperately to remember the name of what I was trying to say)... superior vena cavas!  That's it!  And he got this look on his face and said, "Oh does she now?" And then he turned to her and said something like, well aren't you a little creation or challenge or something like that.  


Here's a diagram so you can see where the SVC is:

The SVC carries the deoxygenated blood from the upper half of the body back to the heart.  It is one big vein, as you can see.  Harlie has two small SVCs instead.  I'll have to dig up some of her diagrams that the surgeon and cardiologists drew so I can remind myself what her SVCs looks like.  


I have no idea how this ties into everything.  But, I can tell you that he felt for a pulse on her right side and could not find one.  And considering he needs to connect the vascular tissue from her leg to blood vessels in her face, this is a concern.  So, he said that they are going to need a CT scan with contrast so they can see what's available and where it's located before they go digging around in there (so he doesn't hurt facial nerves, etc.).  


They also want molds of her teeth.  Oh, and speaking of her teeth, one of the docs was a dentist and he examined her mouth.

Dr. Shusterman examining her teeth.

She really was unbelievably cooperative.  I can't believe she wasn't all shy acting in front of all those white coats!  I really wish I could have taken a picture of all of them sitting there at the table.  I am really surprised I wasn't more nervous talking - but I guess when you're thrown in there isn't time.  Plus, I do happen to know what I'm talking about when it comes to her.  The first thing Dr. Padwa asked when we sat down was, "So, how's she doing?" I tried to stick to what was pertinent to them.

Anyway Dr. Shusterman (the dentist) said that her teeth looked good, considering.  No cavities!  But I know that's because she's never had anything sticky and sweet (like fruit snacks, etc.).  Her molars are coming in outward toward her cheeks because there's just no room to come straight up like they're supposed to.

Dr. Padwa said that their practice would not have operated on her so young (her first jaw reconstruction was at 20 months old) because they just don't believe there is enough bone yet.  And she said the sooner you start jaw surgeries, the more you end up having to do over the long term.  She will have to continue to have jaw surgeries since her mandible will not be able to keep up with the growth of the rest of her face.  She mentioned distraction for potential future procedures.  I won't get into that now, but I've been trying to avoid that for years.  It hurts to think we might not be able to avoid it in the future.  But, we'll just have to cross that bridge later.

Oh, when she asked me, "Her first jaw surgery was at 20 months, right?"  And I couldn't remember really. So, I asked her if she had my medical summary in our file.  She pulled it out and it had highlighted parts all through it - so I knew they had studied it.  Which, I think is really good.  Wow.  Doctors who study the charts before asking 20 questions?  Impressive!

Dr. Meara was the one who asked about her eye. I told him that I just had not been able to research surgeons yet.  It hasn't made it to the top of my priority list.  Which is funny considering I wanted her eye fixed before ever bringing her home!  But then she couldn't even close it at all - which was really freaky. But, we got used to it and eventually she got strong enough to close it and blink it and all that good stuff.  So, it's really not been a priority.  That's when he said they could take care of it at the same time.  Bonus!

Okay, this has been FAR too long.  I will write more about the trip and post pictures in the next post.

Thanks for all your support!
~Christy