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

No Sleep Study!

Harlie has pneumonia.  So, no more waffling about the sleep study scheduled for tonight.  My Mom came over yesterday to watch the boys so I could take Harlie to the ER and she called and cancelled our appointment for me.  I'll call to reschedule later.  It's really so typical.  And I didn't want to go anyway.  ;-)

Yesterday at school Harlie was really tired.  And if you know her at all - you know she's NEVER tired.  Like sleepy tired, I mean.  She might tire from running around and need a little break.  But, she hasn't napped in YEARS.

Terri (her nurse) said that she had to carry her to class.  I dropped her off and I just thought Terri carried her to the sidewalk.  Terri has to carry a lot of stuff.  She's in school herself, so she has her own backpack with her school work, and then she has the suction machine (which is over 5 pounds), Harlie's backpack (which she's not allowed to carry herself because of her spinal fusion) and Harlie's lunchbox.  To carry Harlie (who weighs 34 pounds) in addition to all that stuff is HARD.

She barely made it through circle time with her general ed class, but she participated more in her hearing impaired class.  Then when they went outside for recess, she laid down on the bench.  So, Terri took her inside her classroom to lay down.  Her teacher asked Harlie if she wanted Buster Bear.  NOOOO!!!!!  No more Buster, please!

Harlie said no.  Smart girl.

When her classmates came back in from recess, they went to art class.  Harlie fell asleep in there, too.  So, Terri called me and told me what was going on.  By then she had taken her into the clinic.  She slept in there until I came to get her.  I carried her to the car and brought her home.  I've been meeting Terri at school lately, so she doesn't come home with us (since her car is there).

She was on Day 7 of an antibiotic and she was getting worse.  I haven't seen her this lethargic in a long time.  So, I called Jennifer (another one of our nurses) and asked her about her symptoms.  She stopped by and listened to her lungs.  She listened for a long time and said she thought she had pneumonia.  She said she could hear fluid in her left lung.

So, I called her pediatrician and told him that.  He said to go to MCV's ER for x-rays.  So, I called my Mom and she came over to watch the boys so I could take Harlie.  When I got Harlie up from the couch to put her in the car - I had to carry her like a baby.  She did not even want to sit up to sit on my hip!

We got there and the doctor listened to her for like 30 seconds.  He said, "She sounds clear, but we can get a chest x-ray just to rule it out."  Of course I had to explain her lack of a fever.  Because that's always the first question I get asked - does she have a fever?  Well, no.  But, she never gets them, so please don't let that affect your judgment.

So, hours later, x-rays were taken and read.  Pneumonia in both lungs.   So, kudos to Jennifer to being able to hear what the doctor didn't!

They gave her a dose of Lasix to help her get rid of some of the fluid.  And they put her on a different antibiotic and gave her a dose of that, too.  Then they let us go home.  It was about 10pm by then.  She kept signing "car."  She was ready to go!  And when we left, she had a spring in her step and a big smile on her face.  I would give anything to know what she was thinking.  If only we could have a conversation...

Which reminds me...

At one point the doctor came in and asked me if it was okay if a med student came in to see Harlie because she had never seen someone with Goldenhar Syndrome.  I said sure.  But, it was a little weird to hear him talking to her and pointing out some differences in Harlie's face.  I'm starting to wonder what Harlie understands and thinks when she hears people talking about her, or her peers asking questions about her.  So, again, if only we could have a conversation.

Anyway, she's happier today.

She's home, of course.  And not complaining or whining or asking for anything (other than movies).  She's such a good kid.  Really.  She cooperated with everything and everyone at the hospital.  And she couldn't be any cuter when she gets x-rays.  She follows the instructions and holds really still.  She's something.

Well, that's it for now.  More later!

Thanks!
~Christy

Plastic Surgery Appointment

Tuesday proved to be a crazy day.  I need to back up slightly first.

Sometime in the last few days Murphy stepped on a lego (a constant hazard around here) and cut the bottom of his foot.  Tom washed it and put a bandaid on it, and I forgot all about it.  Until Monday night, when Murphy started complaining about it (or Tom saw some drainage on his sock, or something.  I know, ewwww, right?)  So, we took a look and said, "Hello, Infection!"  There was pus (such a yucky word), his foot was swollen and there was a red line creeping up his foot starting at the site.  Ugh.  Of course, he didn't have school on Monday and I took them to the mall to play - but did he say anything then - like when the doctor's office was open?  No.

So we are supposed to leave the house at 9am the next morning, and I have no idea when we'll be back.  I didn't think he could go another whole day without antibiotics.  So, as soon as the office opened (8:30) Tuesday morning, I called his doc's office and they said to bring him in asap and they would take him and get him back out the door to try to help accommodate our schedule.  They are so awesome.

Tom left and went to get Murphy from school while I stayed home to feed Harlie and pack the car.  Despite our major efforts, by the time we put gas in the car and dropped the prescription off at the pharmacy, it was 9:30 by the time we were on the road.

More than an hour later (we were in Newport News by then) I switched out a movie for Harlie.  And in doing so, I noticed that the floor looked pretty bare.  Then I asked, "Where's Harlie's suction machine?"

Holy Crap.

WE LEFT HARLIE'S SUCTION MACHINE AT HOME!!!!

As the magnitude of this error sunk in, Tom started being funny (our go-to plan for very stressful times). And then I laughed and cried at the same time.  Oh, so many emotions!  We thought about our last thoughts as we were leaving the house.  Tom said, "Don't forget your Diet Coke!"  Thank God we remembered that!  Whew!

And then I remembered plugging it in to charge because somehow we forgot to do that the night before. So, I plugged it in until it was time to go.  While I was loading the car I thought about putting it in the car then, but then thought, "no, what if we need it before we get in the car?"  I didn't want it to be in the driveway while I was in the house because that was too far.

That freaking suction machine has been at Harlie's side every minute of her life (practically) since the day she first left the hospital.

What if she had a plug?  Well, she's certainly had them - but they've not been emergent so far.  But I know our luck.  The day we don't have the suction machine IS the day we're going to need it.  Like really need it.  And since it helps keep her airway open - the thought that I don't have it around is not a good one.

We were WAY too far to turn around.  And she has a much stronger cough now.  And we didn't have a choice.  We were just going to have to be without it and hope for the best.  I thought if I make it to old age, it'll be a miracle.

So, we get to the hospital and see an ENT first.  He said that Harlie's ear is full of fluid, her eardrum is retracted (meaning pressure!) and of course, it's all scarred (nothing new there).  Dang it!  But, it's not infected.  Of course she just had her last dose of antibiotics the night before.  So, who knows what will happen now.  So, to her - it sounds like she's underwater.  Which would explain why we haven't seen an improvement since the blood clot came out a few weeks ago.  Which reminds me, I don't think I ever told you about that.  Ugh.

Okay - real quick... you might remember in this post I talked about how the pediatrician looked in Harlie's ear and then it started bleeding?  It bled off and on for several days.  Then a few days later she started pointing to her ear and signing "broken" which she does when the hearing aid battery has died.  But, the aid was working fine.  So, clearly something was amiss.   I called her local ENT and they worked us in.  The doc pulled out a HUGE blood clot that was blocking over 80% of her canal.  No wonder she couldn't hear!  And that's been in there everyday since before school started!

We thought for sure we'd see a turnaround in her listening and participation at school.  But, after a few days, it seemed to get even worse!  So, the fluid that's in there now would explain that.  The ENT that we just saw yesterday said that if it doesn't clear up in two weeks, we should get her another tube.  Ugh. It looks like I should really look into a bone anchored hearing aid (BAHA) for her.  At least that would give us some back up that wouldn't be affected by fluid or blood, or whatever.

After we saw the ENT, her plastic surgeon came in.  We spent more time with him yesterday than we ever have.  He looked at her CT scan.  I need to figure out how to get a photo of some of the images so I can show you.  Her jaw is crazy.  Unfortunately, my Mac computer won't read the disc.  I'll see if Tom can work on it for me.

Anyway, he talked about what he needed to do.  Her ramus is in a horizontal position instead of vertical, like in this picture:

And she's missing the condyle on her right.  And I think there's something wrong with the coronoid process, but don't quote me on that.

It's really hard to picture the ramus in a horizontal position.  But, her jaw looks nothing like that picture.  She has no angle at all, really.  But he said he could add one in when she's older.  Oh goody, more surgeries!  And her bottom teeth are way off from her top teeth.  Although you don't need a CT scan to see that!  And clearly, the two sides of her jaw are completely different.  Her last reconstruction was done in December 2009.  The bone graft on the left side took just fine.  But the one on the right got infected and had to be removed.  This left her jaw even more asymmetrical than before.

Basically, he's going to have to cut up her jaw into several pieces and then put them back together again in a better way.  If you've ever had any kind of dental work done at all - I'm sure you can only imagine the pain when recovering from this kind of surgery.  It is truly horrifying.

The good news is that he thinks she has more bone to work with this time around.  I guess the bone has gotten thicker as she's gotten older.  So, hopefully that will help a lot.  And he feels confident he can make the changes necessary for this surgery to be successful.  But, we've thought that each time and here we are.

The bad news is that plastic surgeons (especially reconstructive for anatomy defects) are like artists.  Each patient is completely different.  The results vary widely and cannot always be predicted.  And since her structure is abnormal to begin with, it makes it almost impossible to know what to expect.  All these are my words by the way - not his.  It's just my take on it after all these years.

And he said that with Goldenhar Syndrome, not only is the bony structure affected - but so is the soft tissue.  And if the soft tissue won't stretch or accommodate the changes made to the bony structure, then the results might not be favorable.  The soft tissue can actually force the bone to retract or move by the constant pressure.  Which is why it takes a year to know if the surgery was successful.  The agony!

He said that he could do the surgery whenever we are comfortable.

After talking it through, we decided that we would shoot for May, 2012.  Since her jaw has to be wired shut for 9 weeks, if we waited till after school let out for the summer, we would run a huge risk of her not being able to start school again in the fall.  Since she's had 2 out of 3 bone grafts get infected and require additional hospital time, we would rather be safe than sorry.  And Tom was thinking that it would be better for her to miss the last month of her first time through kindergarten, than miss her first month of real kindergarten.

So, if we are lucky, and all goes the way we want it to, we would be hoping for decannulation (getting the trach out) sometime in the summer of 2013.  So, she would be 7 and starting the first grade (assuming she does kindergarten twice, and is able to progress to first grade).

It's really overwhelming to think she'll be almost 7 and still be trached.  I had no idea it would be like this.  And what if this surgery isn't successful?  I can't even think about it.

Which brings me to my struggle with this whole mess.  I was thinking that we should get some other opinions.  I just thought that we owe it to Harlie and to ourselves to make sure that we are making the best decision on this.  So, I spoke with her ENT in DC about it several months ago.  He gave me the name of a surgeon in Jacksonville, Florida.  I e-mailed him today.  And he replied, and in his reply he "strongly recommended" that I reach out to another surgeon in Miami.

And his recommendation got me thinking that I could probably reach out to a dozen plastic surgeons and that I would still be in the same situation.  How will I know who's the right one?  So, thinking things through....

1.  If I went to a different surgeon, they would be going into her jaw with no prior experience as to what worked or didn't work in the two previous surgeries.

2.  I do believe that no matter who performs the surgery - the same issues are present (the question about the soft tissue, bone grafts, results, etc.).

3.  I have to have a facility that has a cardiac anesthesiologist and can support her cardiac issues (or at least get her to DC if needed).

4.  If we travel far away for surgery (like Miami or Boston) how do we do the follow up appointments?  And what if she gets another infection like before?  What if time is of the essence???

5.  I've already seen two other plastic surgeons (in addition to her current one) and neither of them were candidates to perform surgery, in my opinion.  Both of them only perform jaw distraction - not jaw reconstruction.  Her current surgeon does both procedures.  And it is very clear from her CT scan that jaw distraction is NOT an option for her.  Period.  Her jaw needs WAY more than just lengthening.  It needs full reconstruction.  That's just the way it is.  And both of those surgeons were trying to tell me that distraction can be done in almost every case.  Whatever.  I just don't believe that.  And to be fair, they had not seen her CT scan when they said that.  But, the fact that they would say that without seeing her CT scan is concerning, if you ask me.

6.  I am very comfortable with this surgeon.  I think he's a great person, as well as a surgeon.  But, there is a part of me that says we have ONE more shot to get this right.  It HAS to work this time.  But are more opinions going to help or confuse?  I don't know.  But I do know that the thought of something going wrong and having to travel great distances to see her surgeon is VERY SCARY (considering how awful our last experience was, which lead to this).

If this surgery is successful, our lives will change dramatically.  Without the trach, Harlie could learn to speak at a much faster pace.  Her voice would be stronger.  And she would be able to make sound with a lot less effort - which would greatly speed up the process.

Eating could be a lot easier.  I have to say that after seeing her CT scan - I have to wonder how much the alignment of her teeth affects her ability/desire to eat by mouth.  Her bottom teeth fall to the right and are no where near lining up with her top teeth.  So, as I sit here and make my jaw go to the right and then try to swallow, it's damn near impossible!  Try it.  I don't know how in the world she does it!!!  And now I feel like a total horrible mother for making her!

And forget chewing!!!  When is she going to be able to learn to chew???  I really don't see how chewing is going to be an option at all - until her teeth line up somewhat.  And the thought of going more and more years with no chewing makes me want to barf.  Can you imagine the impact of that socially?  Not to mention YEARS and YEARS of more pureeing food for her!!!  Oh brother.  I have to stop thinking about that.  For real.

So, I guess you could say I have a lot on my mind.  As much as I want this surgery to happen, I dread it. The thought of her waking up afterwards and looking me with that look.  The look that says, "What did you let them do to me???"  I just have to hope that the next seven months will provide some growth in maturity and focus to allow us to try to talk to her about it so she'll know what's happening.  Or at least know more than she usually does before a surgery.

It makes my heart hurt to know what lies ahead of her.  Until then, we'll laugh as much as possible.

Oh, and just in case you were wondering, we did just fine without the suction machine.  We didn't need to use it once.  And what a difference it's absence made to our load when walking around the hospital!  And all those times I take it into the grocery store, or Target or wherever?  Maybe we can lighten our load a wee bit?  I think if it's in the car and we can get to it quickly if needed, it would be fine to not carry it all the time.

Well, that's it.  It's super late and this post is super long.  As always, thanks for reading!
~Christy

Ronald McDonald House Fundraiser

I have to give you just a little background info before I can tell you about last night's event.

First - A few years ago, Tom's company moved locations.  During the process they had some leftover computers.  Tom remembered that the Ronald McDonald House (RMH) in DC was in need of some computers.  Since he couldn't get to DC, he called our local house and asked if they wanted them.  They did, so he took them there.  While there, the manager gave him a tour and mentioned that they were going to remodel the kitchen.  Tom said he would be interested in helping them in any way possible.

A year and a half later, he got a call asking if he was still interested in the kitchen renovation.  Of course he said yes.  Tom was invited to bid on the project.  But, it wasn't as simple as a bid sounds.  It was a long process of interviews and meetings.  There was a committee assigned to select a company for the job and that meant a lot of work, and time.  After several months they narrowed it down to three companies.  Then two, then one.  And they selected Tom!  I meant to blog about it weeks ago - but as you know - my blogging time has been scarce lately.

Since then, Tom's been at the RMH several times meeting his subs and going over measurements and plans, etc.  So, last week, Tom came home and told me that the RMH was having a fundraiser the next week and would I like to go.  I said sure.  Then a few days later (Thursday) he calls me and tells me that we have been invited as guests - and they want me to SPEAK about what the RMH means to me.  

Oh, and by the way, it's Monday night and they are expecting about 350 people.   Gulp.

So, I went home and started working on what I would say.  Amazingly, the words just flowed and it practically wrote itself.  

I felt MUCH better once I knew what I was going to say.  

So, on Sunday we had my family over to celebrate Harlie and Cooper's birthdays.  While they were all here, I asked them if I could read my speech to them to practice.  

I read the second sentence (keep in mind the first one was "Hi. My name is Christy".) and started crying.  Oh, no.  That's NOT a good sign!!!!

Everyone laughed (more in support, but also because it was pretty funny and pathetic) including me.  That just made me even MORE nervous.  

It's one thing to live your life - it's another to hear your life in spoken words.  I remember the first time I said that Harlie had Goldenhar Syndrome out loud.  It was hard.  

Anyway, I practiced and tweaked it some.  The impending doom of having to speak in front of so many people pretty much consumed me.  

Finally Monday night arrived and by then, I just wanted to get it over with.  Tom said that they told him that I would be speaking early in the evening, so at least I could get it done and then enjoy the night.  

The event was at a local hotel.  Once we got there (around 6:30) and found his contact, she told us that I would be speaking sometime between 8:46 and 9:00!!!!  Ugh!  The agony!!!  So, we walked around and ate some food (they had a lot of chefs from local restaurants there so you could try their food).  Luckily, it was open bar.  So I had to have a drink.  Or two.

Ronald McDonald "himself" was there, available for photographs.  So, Tom and I went and had our picture taken with him.  It was funny.  I hope they actually send us the picture.  And speaking of pictures, I am so mad I forgot my camera!  Ugh.

Finally, it was time for the speakers.

It says "Tom and Christy" but it was really just me.

My heart was pounding I was SO nervous!!!

I had to go up on stage (with lights!), stand behind a podium and hold a microphone.  Geez.  It was scary!  But, once I started speaking, I was fine.  I really was!  I made sure I didn't speak too fast.  I didn't mispronounce anything.  And I looked up and around the room as I spoke.  I was really proud of myself!!!  

Here's what I said:

Hi. My name is Christy.  

I knew that the Ronald McDonald House helped families.  I just didn’t know it would help mine. 

To give you a little background, we live here in Richmond.  I’m from here, and my husband is from Pittsburgh.  Our experience with the Ronald McDonald House is in Washington, DC.  Our daughter was born there, because we knew she needed specialized care that was not available here in Richmond. 

Harlie turned five years old, yesterday.  A huge milestone, considering during my pregnancy we were told that we had only a 5% chance of ever bringing her home. 

She has a craniofacial syndrome called Goldenhar.  Her abnormalities prevent her from being able to breathe through her mouth and nose, so she is trached.  She’s hearing impaired, she signs and is now learning to talk.  She is a happy, normal little girl with a lot of medical challenges. 

In addition to her craniofacial abnormalities, she also has a series of complicated heart, lung and spinal defects.  She’s had 20 surgeries so far (four of them open heart), and she’s had another 15 procedures under anesthesia.  We don’t know how many more are in her future.  But we do know that she will need a heart transplant at some point - to quote her surgeon - when her heart runs out of gas. 

All in all, she has spent well over a year of her life in the hospital. 

So, we’ve spent a lot of time in a Ronald McDonald House.  It has certainly helped our family financially.  Hotel rooms in Washington, DC aren’t cheap or conveniently located. 

And food is a big issue.  The only option for food at the children’s hospital in DC is their cafeteria.  It’s expensive.  And it gets pretty old after a while.

Because of the Ronald McDonald House we were able to have a healthy breakfast at the house, pack our lunches and snacks and take them to the hospital.  And after a long, tiresome day, we could come home and have a home cooked meal for dinner.  And most nights, cookies or brownies - my favorite.

But aside from all that goodness, the Ronald McDonald House did a lot more for me…

Harlie’s birth was not a joyous occasion, like it should have been.  While we knew about her heart and lung defects, we had no idea she had Goldenhar Syndrome.  When I was discharged from the hospital, I didn’t have to pack up any congratulatory flowers, or balloons.  No one knew what to say, no one knew what to do. 

Our social worker at the children’s hospital told us about the Ronald McDonald House and got us set up there.  We left the hospital and went to Target.  Normally, one of my favorite places in the world, just not that day.  I remember thinking I should be at home in my pajamas holding my little baby.  But, instead I was two hours away, standing in a Target. 

And I had never felt so alone.  And so scared. 

We bought some necessities and headed to our home away from home, the Ronald McDonald House.  I had never been there before.  The manager showed us around, and explained how things worked.  I saw other families there.  I saw thank notes from happy families who had gone home.  And I saw other new moms, who were just like me – confused, scared and in shock.

And then I realized I wasn’t alone.  I wasn’t the only scared mom who had to go through something I didn’t want to go through. 

And that brought an unbelievable amount of comfort to me.  And hope.  And at the time we needed both desperately. 

The next morning I walked into that hospital a new woman.  I had hope.  I loved my daughter and was willing to do whatever I had to do to get her home – where she belonged. 

Now, when we go to the Ronald McDonald House, we take our daughter with us.  The first night we stay together, the three of us.  She plays and explores and has fun.  And then we get up early the next morning to head to the hospital for surgery.  The Ronald McDonald House provides a routine.  It provides comfort – but now not just for me and my husband, but for Harlie, too. 

And for that, we are so, so grateful.  Thank you.

They clapped and I left the stage and went and stood next to Tom.  Immediately people started coming up to us, saying nice things.  They said thank you for sharing your story.  They asked how she was doing now.  And they said that I didn't seem nervous at all up there.  Crazy!  Oh, and two people told me that I just made a lot more money for the RMH.  haha!  

I met two women who lost their sons to complications from congenital heart defects.  One was 10 years old and the other was older than 6.  He died while he was waiting for a new heart.  It was a reminder that even though Harlie is doing great - there are never any guarantees.  Since Harlie's birth, I've met/known so many moms who have lost their children because of heart defects.  It is so sad.  And scary.  As one of the moms said, "they can't save them all."  Truly heartbreaking.  

Another person who came up to us was Ronald McDonald.  And a restaurant owner came up to me and told me that he's had two children born prematurely and he thought he had been through it - until he heard our story.  Then he gave me a hug.  It was really great to receive such warmth and kindness from complete strangers.

I am so glad I had the opportunity to speak.  I think it was a great experience.  And it wasn't nearly as bad as I thought it would be.  

Okay, that's it for now.
Thanks!
~Christy