Monday, February 27, 2012

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!


Just Diane said...

Keeping you all in my prayers. I am thankful for modern technology and modern, forward thinking doctors. You seem to be in good hands.
Are you going to be doing any kind of fundraisers? I am terrible about raising money but I would love to donate to your operation fund. <3

Christy said...

Diane - funny you ask about fundraisers. We've had a few people mention some sort of benefit to help raise some money. Some of my running friends mentioned a running event, which would be exciting. So, we'll see what develops. That is very kind of you to want to help. And that warms my heart. Thank you!

Ann said...

I love that you have a plan and that you are confident with the Boston Children's team - and man, what a team. They certainly get the "customer service" part of healthcare. They clearly respect the time and expense you put into seeing them and it sounds like they made your trip worthwhile. It's a big bummer that they don't take Virginia Medicaid. But, you've got an incredible network of friends that will help you raise the money you need to see this team of doctors. In the meantime, I fear you are going to have to make one or two more "quick" phone calls to see what your insurance and/or Medicaid can do to help with the costs. I'm thinking you should have a six pack in hand when you sit down to make the calls.

Love ya,

B-Mama said...

This is all amazing--You mentioned the long post and I just want to say that I ate up every word of it. Knowing all that Harlie is going to benefit from a surgery like this and hearing about all the positives?! Sign me up! Fantastic news. I will help fundraise however you need help--let's have a fun benefit and race! How cool would that be?? GG and I would totally be game!! xox

Lisa B said...

Glad you had a good hospital visit. It sounds like they're a good team.

I'd be willing to make a donation to the fundraising if you go that route.

Susan said...

Wow Christy! That is quite an appointment. I'm so glad things went so well. I would talk to their insurance department to make sure they wouldn't enroll. Since these are big $ surgeries perhaps they would. And then inquire about a stop-loss on your policy. Many plans have a limit of X thousands and then everything is covered. But it may not apply to out of network hospitals. I think it will be worth a fight.

Good job Harlie! I think she deserves a special treat for making the trip so easy. Though I guess she got the fish, right? So cute she found a stuffy to love.

Tanya said...

It was my pleasure to meet you. I have *never* done anything like that. I am really shy, really I am. I'm very excited that you've got a plan.

I am relieved that they were upfront with you about the costs of the surgery. Hundreds of thousands is not an exaggeration at all.

Christy said...

Thank you all for your support! It is overwhelming to think of planning, preparing and then actually going for surgery so far away. I can't tell you what a difference it makes to know we have so many people who love us and want to support us in any way you can!

Thank you! xo

Robin said...

Christy, it's great to read all the details you wrote about in this post. I even learned some more about Goldenhar and you wrote the best explanation I've heard so far about the "blood flow interruption" that may cause it. THANKS!

We just learned today that we may be changing surgery plans for Faith from a jaw distraction to a scapula free flap - pretty much the same as the fibula, but using the scapula instead.

Best wishes to you all next week with Harlie's pacemaker surgery. I completely understand your feeling about it getting harder instead of easier to put our kids through these things and feeling more scared than we were before! I'm feeling the same way as we get closer to Faith's next jaw surgery.

BTW, If the Tanya you met is the one I know through the Yahoo groups I'm totally jealous that you got to meet!! But very happy for both of you. One of these days we will get to meet in person too!