Post-Op Day 1

Funny that Post-Op Day 1 is also Discharge Day.  I suppose there's a first time for everything.  

We are back at the hotel now.  And while Harlie is smiling in the following photo, she is GRUMPY.  Whew!  The swelling is getting worse.  Tomorrow should be the worst day and then it should start to get better after that.  You think I would have been prepared for that, wouldn't you?  But each time we do this, I'm always shocked.  I guess I block this part out of my memory.    

Health wise, I think she's doing well.  We took the Vest to the hospital and were able to keep her Vest treatments up, which I think helped a lot.  We can't get the incisions wet for 48 hours.  But, her hair had so much blood and goo in it that I had to wash her hair.  So, I gave her a quick bath (without getting the incisions wet) and she protested.  A lot.  I felt so bad because I so wanted to give in to her and not make her take a bath.  But, I couldn't live with her hair the way it was.  She HAS to feel better now.  Right?  

The only thing that makes me a bit nervous is that she has been bleeding a bit since we left the hospital.  I'm thinking it's from the trach collar since she's been moving around a lot more now that she's not in a hospital bed.  

So, since I forgot about the swelling, I forgot that she wouldn't be able to swallow.  Again.  Sometimes, I think we are being tortured.  We get through something so horrible, just to have to go through it all over again.  And again.  Sigh.

Anyway, as far as how the surgery went... it went well.  She said that the new bone that grew looks good.  There's no sign that the infection did any damage.  But, just to be on the safe side, they are leaving her on the antibiotics for one more week.  

So, as far as her jaw goes, we are done for now.  Her surgeon wants to see her next summer.  But, that's it.  Now to get this oxygen thing figured out... 

Anyway, here are some pictures from the last few days...

Harlie and I right before surgery.

They let me go with her into the OR.  She no longer needs Versed to help her go without being upset.  She just goes.  She cried a little bit right before we left and I asked her if she was scared and she nodded her head yes.  I just told her that it would be a quick one and that we'd be right here when she woke up.  That seemed to do the trick.  It was a quick one, so hopefully I am building up some trust with her.

I had a hard time sleeping the night before surgery.  I was so, so tired that day.  And since the Red Sox were playing that night, I knew it would be another long night.  So the second she got moved to the cardiac intensive care unit (CICU) and I saw that "bed" I jumped at the chance to close my eyes for a minute.  Or an hour and a half.

Being in the hospital will suck the life right out of you!

We brought Harlie's Vest and did her chest PT.  This was the night of the surgery and she fell asleep while getting it.  So, I guess it can't be that bad.

This was the morning of post-op day 1...

Post-op day 1

On Tuesday, we went to the aquarium.  It was great!  There was hardly anyone there so it was very comfortable.  It's the longest she's ever lasted there.  And I know it was her best time there.

Watching the sea lions and seals.

And for the first time she actually touched stuff!  Every time we've ever been it has been so crowded in the touch tank area, that Harlie didn't want to go in.  Well, this time, there was plenty of room.  And the girl could go and get things for her to touch so Harlie didn't have to move all around.  Harlie touched everything!  Some things she needed a little coaxing.  She touched an urchin, a crab, and a starfish.  And then we went to the new stingray/shark tank.  And she touched a stingray.  It was so cool to see her having such a good time.

The top of the big tank.

After the aquarium, we went and had lunch and drinks at a restaurant nearby.  Then we went to the carousel.  Harlie really seemed to enjoy it.  I loved that she loved it, but it made me sad.  All I could think about was how much I wish that her life could be more of those experiences than hospital ones.  This was surgery #40.  I think I might be getting tired of doing this to her.  I think we are all ready for a break.

The night of her surgery was the big Red Sox game in Boston.  We went to dinner and watched the game from there.  The restaurant was about three blocks from Fenway.  It was a lot of fun to be in the middle of such excitement!  Oh, life in a big city.

The bartender opened up a bottle of champagne and I took a hit right into my eye!  It was still fun, though.

The streets were packed.  They actually barricaded all around Fenway so you couldn't walk closer.  If you look in the background of the next photos you can see the building in the background with "GO SOX" in lights.  Pretty cool.

That's it for now.  We are flying home tomorrow afternoon (Friday).  I'm a little nervous about people staring at her tomorrow since it will be the worst day of the swelling and bruising.  But, we'll get through it.

Thank you so much for all your kind comments, posts, thoughts and prayers over the last few days.  It was so much fun to see all the photos on Facebook.  Thank you for taking the time to do that for us.  We are feeling the love and it is helping us stay strong.

Much love,
Christy xo

Pre-Op Day

What a long day.

We are beat.  I think Harlie fared better than we did.

Our first appointment was in Pre-Op/Admitting at 10 o'clock.  They did the basics - weight, height, temp and blood pressure.  Then we spoke to a nurse at length about Harlie's history, current status, etc.  After that, we spoke with an anesthesiologist.  She asked why we didn't do a heart cath first.  She was a little concerned about her cardiac situation.  But I explained that it doesn't work that way.  We don't have a choice.  The hardware has to come out first.  And we are here and Harlie is healthy.  I don't know what's going on with her heart and lungs - but she is NOT sick.  So, she sent us on to our next stop - admitting.  Tom handled that one while I sat with Harlie.

It was noon by this point and we had an hour till our next appointment - cardiology.  So, we went down to the cafeteria and got some lunch.  There we ran into Julie, a nurse we used to have when we were at Children's National in DC.  I saw her when we were here this summer, too.  She moved to Boston and now works here.  It really is a small world!  Anyway, it was so good to see her friendly face!

After lunch, we headed up to cardiology.  There she got an EKG, a pacemaker check and we met with her cardiologist who had us this summer.  I find him to be very easy to talk to and compassionate about her and all we have on our plate.  I brought him up to speed on what's been going on with her.

Unfortunately, the conversation wasn't great.  We won't know anything for sure until she gets this darn heart cath.  But, his thinking is that it is not likely to be a simple thing as a collateral vessel(s).  He explained why and it makes sense.  It also is in line with what her local cardiologist has said for years.  Which means that her heart may not be the cause of the oxygen requirement. That will send us back to pulmonary, which means we have to go back to the beginning.  That's where I started my questions last year.  And we never got answers.  She is just so complicated.  Her heart function - the Fontan, is less than ideal, in a kid with normal lung function.  Add her less than ideal lung function to the less than ideal Fontan function and what do you get?  Plus, he said that she could be micro aspirating, which, over time could cause lung damage.  So far, we've never seen any evidence of aspiration, but what the hell?  I suppose she could be.  Nothing about her makes sense, so why not?  But, we've done x-rays, a CT scan of her lungs in June and we are now doing Vest treatments.  I really don't know what else we can do.  Except leave Richmond for pulmonary.  Which I do NOT want to do.  But, I will, if I have to do.

But, I'm probably getting ahead of myself.  We have to take one thing at a time.  But, for now, Tom and I are feeling very heavy.  I don't want this to be a sign of something horrible.  But, the oxygen requirement for almost a full year, the struggle her body had with surgery in July, and then again in August, the addition of Lasix to her daily routine, and then an increase of Lasix every other day, means that we are going in the wrong direction.  Things are getting worse.  And there's nothing I can do to stop it.  I don't understand what's going on, and neither do her doctors.  There's absolutely no comfort in that.  And we have to wait at least SIX weeks for her heart cath!  How am I going to live with this worry for six weeks?

After that appointment, we headed over to Plastics to see her plastic surgeon, Dr. Padwa.  We didn't know how to get there from where we were.  I know how to get to Dr. Padwa's office, but I have to start from a certain place.  Anyway, we bickered about being lost, where to go, etc.  And it was very obvious that we were feeling very tired, and sad, and worried and that's just the way all that stuff comes out - being crappy to each other because there's no one else to do it to.  Luckily, we are not new at this, and we realize what's happening.  So we stop it somehow.

We went to Dr. Padwa's office and met with her.  That went well.  No heavy conversation there.  She thinks Harlie looks great.  And things are so much more improved than when we saw her last.  Harlie is swallowing and her mouth closure is much better.  They got some mouth x-rays and some pictures.  That sounds easy, but it wasn't.  Keep in mind it was well after 3 o'clock by then and we were all so tired.  And trying to get Harlie to look at us and hold still, etc. was a lot of work.  And I can't even say we were successful.  I don't know, maybe.  They said she did well, but all I can tell you is that I was done.  Then we went back towards cardiology to get chest x-rays.  We would have done it while we were right there (they are next to each other) but, we didn't have time.

Here's what she looks like underneath it all...

You can clearly see the hardware in her jaw that will be coming out on Wednesday.  Isn't it crazy?  Oh, my poor sweet little girl.  There is so much going on in her little body.  It makes me so sad sometimes.

Here is a side view...

After that we were DONE.  Luckily, her cardiologist didn't make her get another echo.  And come 4 o'clock (it's scheduled time) I was so, so thankful.  There is NO way she would have been cooperative for that.  And Tom and I did NOT have the energy to help her through it.

We left the hospital after 5 o'clock.  We walked those halls for seven hours, pushing her, and pulling her oxygen, both of us connected by tubing.  Weaving through the halls, and waiting rooms, going into restrooms and exam rooms with that oxygen in tow is exhausting.  I hate to sound like a wuss, but that oxygen changes everything.  And I'd like to think I'm somewhat physically fit and energetic.  Yet, it kicks my ass.

But, in those seven hours, we really never had to wait.  And if we did, it was for a very short time.  Our day in a nutshell:

1.   Spoke with a pre-op nurse.  Got vitals and discussed meds.
2.   Spoke with a different pre-op nurse. Went over history and discussed meds.
3.   Spoke with a pre-op anesthesiologist.  Went over current issues and discussed meds.
4.   Admitting, gave insurance info.  That was easy.
5.   Had lunch
6.   Got an EKG, completed paperwork about her meds.
7.   Got a pacemaker check
8.   Met with her cardiologist
9.   Met with her plastic surgeon
10. Got panoramic x-rays and various photos
11. Got chest x-rays
12.  Realized Tom left his cell in Plastics, so he had to go back and get it

Walking all over the hospital and then discussing Harlie's history, meds and current issues, over and over again - is EXHAUSTING.  But each specialty has to know that the information they are looking at is current and accurate.  So, I get it.  But, it's still exhausting.

Then we left the hospital and went across the street to get some coffee.  Tom went in to order and I stayed outside with Harlie.  Those are the things you have to do when you're lugging an oxygen concentrator around.  Some places are just too crowded for our wide load.  Anyway, while I was standing outside with Harlie, it took all my strength not to burst into tears.  There was a girl standing there asking everyone who walked by if they wanted to discuss the Syrian refugees.  No one wanted to, in case you were wondering.  And I couldn't help but notice that I was standing just a few feet away from her, planted in my spot, and she never asked me.  Funny.  I guess she could tell that I had enough of my own problems to worry about.  Then Tom came out and we walked back to the hotel.  Tom went and got us dinner and we ate it in our room.  Now we are waiting for the baseball game to start.

There is a lot on my mind tonight.  But, somehow we'll get through.  Harlie is happy and that's what's most important.  She was so good today.  She really is a good kid.  And I don't know how or why.  She has every reason not to be.  For now, I thank God that she doesn't understand all of this.

Thank you for all your love and support.  We are feeling it tonight, and we are so thankful.  Also, I want to thank my neighbors, Jasmine and Soloman for having the boys (including Rooney) over for a sleepover with their son on Saturday night.  And my sister, Sandy, for hanging out with them on Sunday and taking them to see a movie (coincidentally, Cloudy with a Chance of Meatballs 2).  And Brandy for staying at our house on Sunday night and getting them off to school this morning.  And, of course, Bethany for keeping the boys and Rooney this afternoon after school until Grandma and Pap Pap got there to take over for the rest of the week.  Whew!  It really does take a village...

Tomorrow is our day off and we are all looking forward to it!  Thank you again!

Much love,
Christy xo

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

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

More Misc. Updates

If you've spoken to me this week, you know I've been stressed.  Kindergarten is hard.  Being Harlie's advocate for her education is hard.  But, I know it will get better.  Everything is just so... new.  The hearing impaired program went through a lot of changes this summer.  The program is new to this school.  The principal is new.  And everyone working with Harlie is new to us.  Our routine is new.  And the longer days and harder work is new.

We are going to have a meeting next week to discuss Harlie's communication issues.  I think I will feel much better after that.  It's just that I know we need to make some changes to her IEP (Individualized Educational Plan).  But, until then, they have to follow the one that's in place.

Also, the bus situation is really stressing me out.

Here's the skinny:  Harlie goes to a school out of our zone that's about a 10-12 minute drive away.  She is the farthest one from the school, so she is the first one to be picked up and the last one to be dropped off.  Our pick up time was 6:48am to be at school at 7:33am so she's in her seat by 7:50am.  Last week it changed to 6:53am.  And then, without telling me, they changed it to 6:41am - so she missed the bus that morning.  Even though it comes to our driveway, she missed the bus.  Then it went back to 6:48am.  It changes because kids are being added or removed from the route.

The drop off time was 2:50pm.

She is spending a lot of time on the bus in a day.  And it is negatively impacting her nutrition.  Today I spoke with Transportation.  Our new pick up time (as of the beginning of the conversation) was 6:40am!!!!  And drop off is 3:00pm!!!  That equals TWO HOURS on the bus each day!  I already wake her up at 6am.  And we struggle getting in an oral feeding for breakfast.  So, we have to tube her a lot in the mornings.

She eats lunch at 10:40am.  She self feeds a few ounces of fruit (at the most) and the rest is formula via her g-tube.  And then she doesn't eat again until she gets home after 3pm!  By the time she gets in, and we get her snack ready, she's eating at 3:30pm.

So, when we sit down for dinner, around 6pm, she isn't ready to eat again.

And she gets a fraction of real food than she's used to getting.  And this has it's own chain reaction of issues.  Real food plus formula works better for her body than just formula.

I explained this to Transportation and she was sympathetic.  But, the fact is that there are only two special needs buses that serve this school (take the kids that aren't zoned to go there, but go there for special programs).  One bus goes to a totally different area of town.  And one comes to our area.  I asked her when they add an additional bus to create another route and she said when the pick up time is 6:30am.

She went on ahead and pushed back the arrival time at school from 7:33am to 7:40am.  So, she changed our pick up time from 6:40am to 6:47am.  She also said she would look at the route to see if they can do anything to help.  But, I don't have high hopes.

So, we might have to drive her there in the mornings.  But, I'm stuck in the afternoons.  Cooper naps and there is no way I'm waking him every day and letting him get shorter naps five days a week.  Plus, picking her up means I'm not picking up Murphy.

And then I think about how different (easy) it is to get Murphy up, dressed, fed and to school.  Ugh.

That reminds me, Cooper is liking his new preschool.  Today was his second day.  And when I picked him up his teacher told me that he needs to work on his "listening skills."  Really?!?  I had no idea.  Is there anyone that doesn't need to work on their listening skills?  Especially a two (almost three) year old?    Funny stuff.

Back to Harlie again, she counted from 1 to 11 (the number of days till her birthday) on Wednesday - using her voice, in front of the whole class!  And her teacher told me that she asked for the "blue lizard" using her voice and she totally understood her!  She is trying to verbalize so much more now!!!

Her teacher also asked me if I wanted to provide a goody bag to keep in Harlie's class.  So, when parents bring in treats (cupcakes, cookies, etc.) for the whole class, she can pick something out of her goody bag so she isn't left out of the celebration.  Awesome!!!

I've started working on Harlie's private therapy schedule.  My plan (as of now) is for her to see her physical therapist and a prior speech therapist (to work on the device) on Thursday afternoons.  I think I might hire an additional speech therapist for another day to work on her verbalizations.  I've got messages out to two STs, so we'll see what we can work out.

I called our local rep for the company that makes Harlie's communication device.  I am going to schedule some time with him so he can train me on some things on the device.  He will also train school personnel, so hopefully we can work something out soon.

I made an appointment to see Harlie's plastic surgeon in October.  She's going to have to miss a whole day of school for that one.  That stings.  But, there is nothing I can do.  He only has clinic one day a month.  He said after the last surgery that he would want to wait years before doing another one.  And by the time summer gets here it will be almost THREE years since her last one.  That is so hard to believe.

We are also going to see her local ENT next week.  Ever since that episode when her ear bled, she keeps pointing to her ear and signing "broken" to tell me that her hearing aid isn't working - even though it IS.  So they worked us in for next week to have her look in her ear and then be tested by her audiologist.  I am hoping it's nothing serious or permanent.  And I'm trying not to worry about it.  But, of course I am!

I forgot that I haven't shown you Harlie's book!  I will take some pictures of it and post about that soon.  They are passing it around to all the kindergarten classes.  Today another class read it and a lot of the kids came up to Harlie on the playground to say hi.  For the most part they all seem to want to play with her.  But one girl wanted a closer look at everything and would not get out of Harlie's personal space.  Brandy tried to answer her questions (why this, why that) but she wasn't satisfied.  The worst part is that while she was turning her head and studying Harlie, she had a scrunched up look on her face.  When she wouldn't stop, Brandy had to send her away.  Brandy said that Harlie didn't seem to notice the girl's expression, she just wanted her to get out of her face.

As smart as Harlie is, I can't help but wonder if she's more aware than she's letting us know.

Well, it's late.  So, that's it for now.

Thanks!
~Christy

We have another surgery date.

Late Friday afternoon, I got a call from Harlie's plastic surgeon's office. She said that Dr. Magee had a cancellation and he could do her jaw reconstruction on, gulp, DECEMBER 9th!!!! We were thinking this surgery would happen sometime around February.

It's just kinda weird to think about it being so soon. Maybe I'm in shock. I need time prepare - mentally. And right before Christmas?! We were supposed to go to Pittsburgh the week before Christmas. Now that won't happen. There's no way she'll be able to travel that soon after surgery.

But, I don't see how we can pass up the opportunity. And I guess it's a good thing that I won't be able to spend the next several months thinking about it.

Yep, that's a really good thing.

Luckily, Tom's mom can come down to help us with the boys. And it's better for Tom's work schedule to do it in December vs. February. So, hopefully that means he can stay with me the whole time she's in the hospital. If I remember correctly, she was in for just about three days last time.

Uh oh. I just realized that she needs to get that G-J Tube before she can have the jaw reconstruction. Hmmmmm. I wonder if I can pull that off. I think they do it radiology? So, maybe that will help? Ugh. That will definitely be the first call I make on Monday.

Anyway, so early this morning my friend, Natalie and I were on our way to meet our running group for our last pre-race run, when we were rear-ended at a red light. We were just sitting there chatting and BAM! There was no warning. No brakes screeching or anything. Which, honestly, I think was to our benefit. We were relaxed when we were hit, which I think is a good thing. His car was pretty crumpled and both his air bags deployed.



Natalie's car appeared to have just minor damage to the bumper. But when we drove away, there was definitely something going on underneath the car. We didn't make it to the run, which I guess wouldn't have been that smart to run right after that anyway.

We are fine, by the way. Just some lower back stiffness, which I really think will be fine in a day or so. Now we just have to hope that nothing like this happens on our way to the race! Missing the race would really be VERY disappointing after all the training we've done!

Well, that's it for now.

Take care,
Christy

CT scans Friday

So, I got some bad news today. I was on the phone for a while today with the Radiology department at the children's hospital in DC. Harlie is scheduled for her CT scans at 8:30 am on Friday. Well, after several phone calls and difficult conversations - it turns out that we have to be at the hospital at freaking 6am Friday morning. Which means we have to leave at freaking 4am! This is going to cause one heck of a crummy chain reaction, that's for sure.

There is so much to this story, it is hard to figure out where to start! Honestly, I feel sorry for anyone reading this post. It might make your head spin.

Plastic Surgery

A few weeks ago, we got a second opinion from a plastic surgeon at the hospital in DC. The plastic surgeon that did her first jaw surgery is in Norfolk, VA. So, Tom and I went and met with the DC guy about Harlie's jaw. But he can't really tell us anything until he sees what's going on inside. Which means that he needs to see CT scans - current ones.

One thing to keep in mind here is that getting a second opinion is not an easy thing to accomplish. It took THREE months to get that appointment. In a perfect world, she would have gotten the CT scans first - but the surgeon needs to order them, and in order to order them, he has to see you first.

Spinal Surgery

She needs CT scans for her spinal surgery. I started working on getting the CT scans scheduled back in JULY! And she will finally get them October 9th.

Now I knew that the plastic surgeon would want them, too. So, I made the CT scan appointment for after the plastic surgeon appointment so he could put his order in and they could do all of them at once, to consolidate her anesthesia time.

In order to truly get a second opinion on her jaw, we'll also need to meet with her surgeon in Norfolk. And he'll need current CT scans, too. So, to avoid having to get CT scans done in Norfolk, I will get a copy of the study done on Friday on disc and will take it to him.

Backing up for a sec, her original CT scan date was Wednesday October 14th. When I called to schedule the appointment with her Norfolk plastic surgeon, his assistant told me his next clinic day (he only has clinic days once a month) was Tuesday, October 13th. The day BEFORE her CT scans! So, that would mean I would have to wait till November to see him. But, he isn't going to have clinic in November because of some other thing - so that put us in December! Ugh! More time wasted!!! I was terribly bummed, but what are you going to do? I scheduled our appointment for December, kicking myself for this whole second opinion idea. Keep in mind that our original plan was for her to have jaw surgery this fall - which clearly, didn't happen.

So, I thought about it and decided it could not wait until December. So, I called and rescheduled the CT scans for Friday the 9th, and then called back her Norfolk guy and she got us in to see him on the 13th. Perfect. Whew.

Today, in my conversations with Radiology, it turns out they had her scheduled for conscious sedation instead of general anesthesia. And as I've been told, general anesthesia is "safer" for her in that she is more closely monitored by an anesthesiologist and better equipment (?). I haven't done much research on this because, quite frankly, there's only so much time in a day. And really, it doesn't matter because if an anesthesiologist tells me it's safer, well, I'm not going to argue.

Anyway, they told me that they didn't think they could get an anesthesiologist on such short notice. Short notice? I started working on this in JULY!!! The scheduler knew about her complexities because she is the one that told me she couldn't have an MRI - weeks ago. Anyway, I tried to stress how important the timing was of these CT scans. If she can't get them on Friday, that will set us back an additional TWO MONTHS!

So, after a while they called back and got everything straight. And that's when they told me about having to be there at 6am. Brandy is going to kill me when she finds out. And I swear, home health nurses just don't get paid what they deserve. But, given the crucial timing, I'm thankful it will still work out, so I guess I shouldn't complain.

Oh, they also told me that she was scheduled for a lumbar puncture (ew!). When I asked why the woman said, "because the patient is complaining of back pain." WHAT? I told her she needed to double check that paperwork, as my daughter just turned three, and she hasn't complained to me - much less to someone up there. She called me back to tell me that she's getting that so they can put contrast in there for the CT scans. Oh, okay. Now that makes sense. All's good. Although the words "lumbar puncture" kinda freak me out. Which is kinda funny considering all the words I've heard and learned since Harlie's birth.

So, Friday is going to be a loooooong day. And for the chain reaction... Saturday morning I will get up early in the am to go run 9 miles. The farthest I have ever run in my life. Exciting stuff.

Then as soon as I get back I will have just enough time to eat, and get ready to go to work. I'm working this Saturday where I used to work - just helping out when I can. I will work 12-5. Then I will come home and get ready to go to my 20 year high school reunion. 20 years. Holy moly. Talk about a long couple of days with lots of stuff packed in them.

And, if you read - and understood - all this, I'm proud of you.

Tomorrow is Harlie's hearing test. I'm dying to know the results. Her appointment is at 3:30.

Well, that's it for tonight. And I said that I didn't feel I was up to blogging tonight. Ha! I'm sure I'll regret it in the morning.

Thanks,
Christy