Monday, September 1, 2014

The eve of the first day of school.

Tomorrow is the first day of school.  Sleep isn't coming easy tonight.  And neither are the words for this post!  I have typed 17 sentences, and backspaced over each one!  I HATE complaining and I'm afraid this post may come across that way.

But, here's what I want to say... tomorrow is the first day of school.  If you are kissing a child good-bye, taking some cutesy picture of them holding a frame, and sending them off to school, with a lunch that they will eat - by mouth - you should take a moment to consider how lucky you are.  If you are amazed at how big your child has gotten, and how much they've grown, you are lucky.  If, when you completed the health form in their back to school paperwork, you got to answer mostly "no", you are lucky.  I mean it, you are truly blessed.

And we are lucky, too.  We have two eager to learn boys, who I will get to walk to school tomorrow.  Murphy is starting 5th grade and Cooper is starting Kindergarten (do you hear the angels singing?).  And I am not sad.  Not one little bit.  They are growing, thriving, easy loving and learning kids.  What's to be sad about?  I knew when I had them they were going to grow up.  The alternatives to growing up aren't good, after all.

And while I know we are lucky to have Harlie (in every sense of the word), it sucks more than I can say to not be able to send her to school tomorrow.

Yes, one could argue, "it's better for her."  And that's probably true.  And, exercising regularly, eating more vegetables and drinking less alcohol is better for you, too.  But does that make it any easier to do?  No.

The bottom line is that she cannot attend school for health reasons.  And you know what?  That sucks.  Plain and simple.

I might have forgotten to mention that back in June when Harlie had her pacemaker adjusted, I asked her cardiologist about her attending school.  I knew in my heart what he was going to say.  But, I asked anyway.  He asked me how she's doing at home, learning-wise.  And she's doing well.  One could make a very strong argument that she has done better at home academically, than she did in school.  So, it's simply not worth the risk.

Medically, nothing has changed from last year.  While she can handle small breaks from the oxygen, her lungs are no better.  And one bad sickness could mean serious consequences for them, for her and for us.  With limited alveoli producing oxygen in there, you can't risk losing any more.

I have to remind myself that this decision isn't mine to make.  I cannot possibly tell you how difficult that is.  Every single day I want to figure out a way to make it happen.  I want her to have SO MUCH MORE.  Keeping her home feels like I've given up.

Some days it is so hard to be her mom.

I just looked back at the last few years of "first day of school" posts.  It made me sad.  So much hope I had.  So much effort we made to get her tiny little butt to school.  And for what?

And, is this forever?  Will she never go to school? What about school pictures?  The yearbook?  It seems wasteful to buy her a yearbook with a bunch of kids that she never even met.  And do I take her to school on the day of school pictures?  The thought of going into that school with Harlie, and it not being for school brings tears to my eyes just thinking about it.  And what about those little frames where you put each year's school picture in it so you can see how they've grown?  It would be mostly empty for her.  Which means I shouldn't get one for her.  Which means I shouldn't get one for the boys, either.  Which makes going for school pictures seem kinda stupid.


I don't even know what grade she's in.  Technically, she's starting 2nd grade.  But, that's kinda bull, because she didn't get any science or social studies last year.  And she still doesn't know how to read.

We went to the pool today and I saw girls that were in kindergarten with her the first time she started (she repeated KG).  They are now starting 3rd grade.  And they are so big and grown up.  And it's like Harlie is frozen in time.  She's still so tiny.  She still struggles to talk (although she is talking SO much more and we are now able to understand SO much more - which is fantastic and never for a moment do I take that for granted, I promise you!).

Anyway, it just feels so weird.  But, like I said, we are lucky to have her.  That is true, and I hold on to that every day.  She is so funny.  And smart.  Even though academically, you might not be able to see it, or measure it.  Just tonight when Murphy and I walked Rooney, he was telling me a story about Harlie saying something.  And he was laughing.  He enjoys being able to understand her just as much as I do.  And he thinks she's funny, too.  Just because it will put me in a better mood, here are a few things that she says that is really funny the way she says them...

1. She gives her baby doll to Murphy and/or Cooper and says, "you take care of the baby."  The other day I was feeding her and she signed "full" and "sick."  I said, "You're not sick!  Mommy's sick."  I was just joking around.  Time passed and I went upstairs and heard Harlie in Cooper's room telling him he had to take care of the baby.  He said, "I don't want to - ask Mommy to do it." Then Harlie said, "She can't.  She's sick."

2. If she asks for something (like to get on the computer) and we say, "in a minute" and more than a minute goes by, she says, "now?"

3. She keeps on telling us that she wants a "pink creature power suit" (from the show Wild Kratts).  The first time she said it I had NO idea what she was saying.  So, I called Cooper in and asked him to translate.  He looked at her and said, "What Harlie?"  She "said" it for the 400th time (1st for him) and Cooper looked at me and said, "She says she wants a creature power suit." and he turned around and ran out of the room.

4. I crack up every time she calls "Boys!" to Murphy and Cooper (like we do).

5. It's both wonderful and sad when she says, "I don't want oxygen."  I love hearing her talk, but hate that she has to have it when she doesn't want it.  The other day Brandy said her sats were 81 (I was in my office and Harlie was in the living room) so she went to go get the oxygen.  When Harlie saw the tubing, I overheard Harlie say, "I don't want oxygen." And then Brandy said, "But you need it." And then Harlie said, "But, I'm 81."  She looks at the pulse ox and looks at those numbers.  She never ceases to amaze me.

6. The other day she had her baby in her hands and she looked up at me and said, "I love her."

Nope.  Not going to take anything for granted when it comes to her.

Like when she made me get down the double jogger so she could take her baby.  How could I say no?

Or the time she tried to hula hoop with the big girls at a pool party.

I love the way she plays with Rooney.  Although, he might not always agree.

Minutes later I saw them having a heart to heart moment. While she was wearing swimming goggles.

And how could I not feel lucky when I get to see this...

Every time she rides her bike, I can't stop smiling.  She works so hard.  And she really is very happy.  I think this was her first summer doctor/hospital/surgery-free.  And she will be EIGHT years old this month.  Isn't that crazy???

Anyway, I will fight the sadness.  And I will continue to choose to find a happier perspective.  For as long as I possibly can.

Thank you for your love and support!
~Christy xo

Friday, August 1, 2014

Vacation in June and an Update

So long without a post!  Ugh!  Our life is full and busy, which is good I suppose.  But, to be honest, blogging about it hasn't been appealing to me for a long time.  I have been in a crummy place for a while now.  And time.  Time is such an issue.  My office has been taken over by Harlie's schooling and the kids watching Minecraft videos.  What in the hell is up with that?  I just do NOT understand that game.  And between my part-time job (granted, very part-time) and it being summer with the kids home, it's extremely difficult to get in front of the computer.

Anyway, I'll just start talking and see where it takes me...

Vacation, June 14-21

We went on vacation to Lake Anna with my family the week after school let out.  Tom had to work most of the week, so he wasn't there much.  Luckily, since it's only about an hour away, I had nursing for the week.

It was so great to spend time with siblings and my nieces and nephews.  They are such a fun group of kids and we just don't get to see each other as much as I would like.  So, that part was great.  The part that wasn't so great was how little Harlie was around us.

When we got there (it was a big house on the lake, with a really nice boat house) we found this float? tube? not sure exactly what it is intended for, but it was perfect for Harlie.  It had a mesh bottom, so it basically became a kiddie pool in the lake for her.

In that picture she wanted to sit in an additional float, just for fun, I guess.  Anyway, this was a perfect solution to keep her cool on hot days.  Although, to be honest, it's hard to relax around water with Harlie.  It's never far from my mind that everything could change in an instant.  Especially in lake water!  Oh, and someone told me after that week that kids with g-tubes shouldn't be in lake water because of the risk of parasites or something.  Awesome.  Luckily, there were no issues.  But, sheesh!  What's a girl with a trach and g-tube supposed to do for fun?!?!

Anyway, we got there on Saturday and she only spent some of Saturday and Sunday in the float.  After that it got too hot for her.  And some dragon flies came out, and she was done after that.  The girl does not like bugs of any kind.  The rest of the week she spent in the house with her nurse.  In the room where she was sleeping there was a TV with a DVD player.  She thought it was pretty cool to lay in bed and watch movies all day.  While that KILLS me, I try really hard to look at it from her perspective, and if she doesn't get to do that at home, I suppose that's what vacation is all about.

The other thing was that the house was not very close to the water.

Here's the view of the house from the dock.

And here's a view of the house and boat house from the water.

We took a wagon to help get Harlie and her stuff from the house to the dock.  But, with the oxygen concentrator, suction machine, etc., it was labor intensive to get her there and back.  And it was a hot week.  And Harlie just can't handle the heat.  Even when she goes into the water, she can't get her head wet.  So, it doesn't have the same cooling affect like it does for us when we can just jump in.  Anyway, it made me sad that she couldn't be with us, enjoying the same things we were enjoying.  And then I felt guilty for spending so much time away from her.

I know she still had fun.  She is always happy, that's for sure.  And I had a great time, too.  I got plenty of down time, and quality time with my family (minus my husband, unfortunately).  And I even got in all of my training runs!  Which I think is pretty impressive considering it was super hot, not good running terrain and I had my fair share of coconut mojitos.

Harlie got to fish, which was pretty much all she wanted to do.

Harlie's first fish!  And the only bass caught that week!

This girl cracks me up.

Dawn and Harlie

Cooper got in on the action, too!
My Mom and Dad got to ride jet skis...

My Mom (taking time off from chemo) and brother, Bruce.
It was awesome to see her have fun!

My Dad.

My Dad and Tom.
We went tubing.  Which is crazy.  Because I am just too old for that crap.

I am way too afraid of hurting myself.  I'm just not into that.  I thought that since I was on the tube with the FIVE year old, I was safe.  Apparently I was wrong.

I mean, look at this!  Poor Murphy!

Okay, maybe he thought that was fun.

Now, here's where it got real.  My new, and only pair of "nice" sunglasses that I've ever owned (that Tom got me for Mother's Day), flew off my head, never to be seen again!  I have included this picture for your enjoyment only.  Not mine.  Just look at the agony/fear on my face!!!

Yes, they told me not to wear them.  Did I listen?  No.  I repeat, I was on the tube with the FIVE year old, I thought it was going to be a nice, leisurely ride.  Clearly, I thought wrong.  

Apparently, while I may be tough on the inside - I'm a wimp when it comes to water sports.  And now I'm back to wearing cheap Target sunglasses.  Oh, the agony!!!

We sat on the dock and watched a storm come in.  We waited till the last second and then ran for the house.

One night for dinner we went via boat/jet ski to a restaurant on the water.  It was a night when Tom wasn't there and I didn't have a nurse.  The boys went on the fast boat, and Harlie and I went on the pontoon boat.  She loved it.

My niece, Jordan, just because I thought
this was such a good picture of her. 

Bruce and Nancy on the jet ski next to us.
When we docked at the restaurant, I realized we didn't have Harlie's chair.  So, she tried to walk.  But it was far.  So, everyone helped me by carrying something (her suction machine, my bag, her oxygen tank) so I could carry her.  It really wasn't that far.  But, it was to her.  And it makes me so sad to see how little endurance she has.  Any exertion at all is taxing on her and she just can't do it.  One second you feel great, enjoying a boat ride, and the next you get a slap in the face at the gravity of your daughter's heart and lung crappiness.  Sometimes it just sucks more than others.

Our last day was Friday.  Tom had to work and could only come out for half the day.  He brought Rooney with him.  So, he got to spend some time on the lake.

Not too far out in the water there was a table and volley ball net.  So, we played a game of beer pong.  Or two.  The under-aged kids just watched, of course.

Me and my beautiful nieces, Kelly, Maggie and Jordan.
Cooper with Poppy (my Dad).

Tom and I with the boys and Rooney.

Doesn't Murphy look so old in this picture?
Skinny.  But, old.

And Rooney liked it, too.

As did Harlie, of course.

I could so do lake living.

When Tom first got there around lunch time on Friday, he pulled into the driveway and him and Rooney came straight to the dock.  Harlie was inside with Dawn.  I was so excited to see if Rooney could swim that we put him immediately in the water to see what he could do.  He can swim, I just don't think he really enjoyed it that much.  So, I took a very short video on my cell phone and we didn't make him do it again.  Then Tom went up and got Harlie and brought her down, so she could see Rooney.

Hours later, we played and packed up and left.  Harlie and Rooney were in my car and the boys were in Tom's truck.  Harlie wanted to see the pictures on my phone (something she likes to do) so I gave it to her as we were leaving the house.  She came across the video of Rooney swimming and started to cry.  Like a legit, "I'm so sad and disappointed" kind of cry.  I turned to look at her and she said/signed, "Mama, I want to see."  She missed it.  She was in the house when Rooney was in the lake.  And she missed it.  And she knew it.  It's moments like these that kill me.  If her body were stronger - she would have been playing outside with the rest of us and she would have been right there.  With little to no effort.  Instead, just playing outside can be too much for her.  UGH!!!  How did this happen?!  Why?!???  Such futile questions.  It just sucks.  Plain and simple.

There's a bunch of other stuff I want to tell you about, but this is already pretty long, so I'll finish with a "quick" update on Harlie, medically speaking.

I can't remember if I already told you about Harlie's decreasing heart rate.  But, just in case, we realized in May that her heart rate was slower than it used to be.  And hanging out around 60 bpm during the day.  Seemed pretty low to me.  Her pacemaker was set to fire if her heart rate dipped to 50 while she slept, and 60 during the day.  If her heart rate was hanging around 60, that meant it was because her pacemaker was doing the work.

So, on June 25th, we went in to see her cardiologist.  He read her pacemaker and agreed, it was too low.  Basically, due to her heart disease, they expected that her heart would need the pacemaker full-time eventually.  Which, is where she is now.  He said that her heart was working on it's own only 12-14% of the time.  I think since she's now at a place where the pacemaker is taking over, he has more room to control it.  They've always wanted her heart to do what it could on it's own, then be assisted by the pacemaker.  It has been a struggle since her heart initiates a beat from all over her heart, at random paces.  So, the pacemaker couldn't predict what her heart was going to do.  Now, I think he can just set her pacemaker to do what is best, and it's pretty much taken over full function.

After making some adjustments, he said to make her walk up and down the hallway a few times so he could see what happens.  Brandy took her so I could chat with him without Harlie being able to hear us.

I want to know how long we have before things get bad.  But, he can't tell me that, of course.  I asked him what I should be doing and he said there's nothing I can do to change the way her lungs are.  Maybe her left lung will be strong enough to support her one day.  Who knows?  But, eventually, we will be at a place where our only hope/option will be a heart and lung transplant.  If only he could tell me when.  He said he would want us to think long and hard before going down that road.  It's a hard road, with a lot of pain for her, and not good outcomes.

I told him I can usually stay positive, but this latest info is kicking my ass.  I guess her doctors could tell that I had so many hopes and dreams for her.  I really thought she - we - were going to conquer all her challenges and one day, live a normal life.  I suppose from a medical professional perspective (who has seen way more than I ever have) they could see that I was living in la-la land.  The odds are just so against her.  There are too many challenges with too many of her body parts.  All it takes is one to go the wrong way...

I have never thought of her conditions as being "terminal."  Except when I was pregnant with her.  I knew the odds were stacked against her then, for sure.  But, it really seemed like after that, she could beat it.  We've never done anything crazy to keep her alive.  Just a whole bunch of surgeries.  None of them insane.  Sure, her leg bone is in her face.  But, even that's just another surgery day at Boston Children's Hospital.

But this?  When we get to the point where we really need to make a decision about this heart and lung transplant.... that seems insane.

And all my hopes up until now were fixable things.  Like, maybe another, different, jaw surgery will be the ticket to a better airway.  After this heart surgery, her heart will be better.  After spinal fusion surgery, her back will be better.  But, now, I have to hope that her left lung will just be able to handle the workload?  Even though right now, it can't.  At 7, and 46 pounds, it needs help.  But, maybe that will change.  Maybe there will be more medical advancements made in time to help her.

That's ridiculous.  How am I supposed to work with that?

So, that's where I am right now.  I need to find hope again.  And I need to figure out what I'm supposed to do when I hear someone say, "maybe she'll be a _____ when she grows up."  How do you live when you don't know how long you'll have your child?  Will she drive a car?  Get a job? Go to college?  I don't think about any of those things when it comes to the boys.  I just assume, that aside from some tragedy, that they will get to experience all of those things.

And for now, we have no focus.  We're not working towards getting the trach out.  We're not working on eating by mouth.  We're not working on ... anything, really.  For seven years, we've been working on things.  And now, we've stopped.  And I feel lost.  I don't know what to do.

When I was young, I worked at a small, family owned restaurant.  I was a waitress.  And if you ever did that, you might know what I'm talking about.  When the restaurant is really busy, you're on.  You have energy, you can remember stuff, you're working hard and feeling good about it.  But, after the rush, when the restaurant is empty and you only have a few tables, you suck.  You forget to check on them.  You forget their drinks.  You have enough down time that you can sit down for a second and then you realize how much your feet hurt.  Getting up again is that much harder.

That's kind of how I feel.  We've been so busy for so long.  And it slowed and I sat down.  And my feet hurt.  And I'm really freaking tired.  And now rescheduling that appointment with nutrition that we missed a few weeks ago seems an impossible task, that has been on my to-do list for weeks.  Going anywhere takes an exorbitant amount of energy.  And that layer of grief that is always just below the surface, is harder to rise above.

I know I have to figure this out.  Living with this sadness, I mean.  Maybe once school starts, it will be better.  We'll see.

Anyway, back to the pacemaker appointment... Harlie and Brandy returned from their walk and Harlie was definitely breathing heavy.  But, her sats were 84 and her heart rate was 120!  To put it in perspective, when we went to her cardiology appointment in February/March, we were in the waiting room and her sats were 90, on one liter of oxygen.  While in the waiting room at the end of June, her sats were 86, on two liters of oxygen.  After walking, her sats were 84 on two liters (but this was after physically exerting herself).  So, that's huge!

Now, a month later, on July 31st, she rode her bike around the block for the first time EVER.

What a wonderful change!  Granted, with training wheels and she went really slow.  We took the jogger stroller just in case she couldn't make it all the way around.  And we put the oxygen tank and suction machine in that and walked beside her the whole way.  I had to push on her back a little to give her a little help when the road was flat or inclined.  When she went down hill she would go two inches, and then apply the brakes.  It took us about 40 minutes to make it around.  But, she did it!  And she never once asked to get in the stroller, so that's awesome!

Happy Birthday Brandy!
I'm thinking that pacemaker adjustment was life changing.  She's had more energy in the past several weeks than she has had in years.  She actually sits and plays with toys instead of watching TV all the time.  I think she had no energy before - so it was easiest to just sit on the couch and watch TV.  Now she plays!  A few weeks ago, the bike got out.  I don't know how.  We got it for her years ago, and it had never seen the street.  But, she rode it around the house.

What?  Your kid doesn't ride her bike in the house while wearing a cat costume?  In July?

Okay, this has been long enough.  I have so much more I could share, but I am running 14 miles in the morning.  So, I have to get to bed.

Thanks for reading!  And thank you, as always, for your support.  Without my friends and family, I would be a mess!

Much love,
Christy xo

Friday, June 13, 2014

My Speech, and a few other things.

A few weeks ago, I got a call from Mandy at The Pediatric Connection, the company that supplies all of Harlie's equipment, supplies and nursing.  She asked if I would come and speak during their annual meeting, July 11th.  I was in the car at the time, driving Cooper and his friend James to their swim lessons at Aqua Tots and, if you can believe this, I was running a little late.  I know, crazy!

Anyway, she said they ask a physician to speak and a family member to speak, and they wanted me to speak as the physician.  Just kidding.  As the family member.  Sigh.  As much as I worry and fret about these public speaking engagements, I don't feel as though I can turn them down.  So, of course, I said yes.

But, it was July 11th, so I had plenty of time to worry and fret about it later.

So, last week, Brandy called Mandy to ask her a question about supplies.  And Mandy ended the conversation with, "See you on Wednesday!"  And Brandy, confused, said, "What's on Wednesday?"  And Mandy was like, "Our annual meeting - Christy is speaking."  And Brandy was all, "Oh, Christy thinks it's JULY 11th."  Mandy said, with a nervous laugh, "No, it's JUNE 11th."


Okay, I have a few days.  I can do this.  No problem.  Tom was headed out of town for a guy's weekend.  But, I had a nurse coming for the weekend, so I was good.  Brandy was going on vacation, but my nurse said she could stay and cover her two days, Monday and Tuesday, too.  Great!

Unfortunately, things didn't work out that way.  Tom left and my nurse had a family emergency that didn't allow her to come as planned.  No nurse - for 5 full days.  And by full, I also mean FULL of commitments.

I scrambled and luckily my friends and family pitched in to help.  I missed the adult pool party on Friday night.  And, if you know me, you know how much I HATE to miss a party!  We just stayed home and watched a movie.  It was good.

Then on Saturday, Nancy, my sister-in-law, came to get Murphy and take him to his swim practice.  I sent Cooper down to my friend Bethany's house.  And I put Harlie in the jogger stroller and tried to get in a few miles.  It was hot and the stroller was heavy (kid, suction machine, oxygen tank, you know... the usual) and I was happy to get in 4.5 miles.

After I got home, I got Cooper and we rushed to the pool.  Hung out there for a while and came home.  After running around (literally) and working so hard to make sure everyone was safe, happy, fed, etc. Cooper whines in the car on the way home, "Awww, I never got to do a belly flop off the diving board."

First of all, who the hell wants to do a belly flop off the diving board?!  Seriously, what is wrong with you, kid?

Secondly, for the record, no one prevented him from doing this (although given the chance, I would certainly try).  He had more than THREE hours to do what he wanted at the pool and clearly, he never chose to do that.  Which, I wish I could contribute to good sense.  But, clearly, he has inherited my poor time management skills.

And, while driving home, exhausted, I couldn't help but wonder, is it ever good enough?  As parents, and especially moms, is all our hard work to make their lives richer (as in good times, excitement, fun, etc.) ever good enough?

The rest of the day was spent trying to get them to stop annoying each other, and most importantly, me.  It was great fun.  Not.

We all survived, and that's what's most important.  But, I never got two seconds of time to myself to write my speech for Wednesday.  No problem, I thought.  I'll have time when Murphy's at school on Monday and Tuesday.  Well, with no nurse, that proved impossible.  I got some time Monday night, after everyone went to bed.  But, not enough to finish it.  Mandy said they wanted me to talk for about 15 to 20 minutes!  This was no quick write.

Again, Tuesday night, I worked on it and finished it.  I felt pretty good about it.  But, was a nervous wreck anyway.  I don't know why I fret about these things so much.  But I do.  I always have a sense of, it's just my life, why would anyone want to hear me talk about it?

But, it was the annual meeting of a pediatric home health company.  They must care about kids and their families!  It's their business!  And, as it turns out, they do.

I woke up feeling really crummy.  My throat was sore and I could not stop coughing.  Every time I tried to take a deep breath, my lungs needed to cough.  Great.  How was I going to get through 15-20 minutes of talking?!?!?

When Jamie got here, I ran up to Walgreen's and got some cough syrup.  I rushed back home, took a shower, got ready, Kelly (my niece) came over to watch Cooper and Jamie, me and Harlie left for the Science Museum.  I never ate breakfast and was feeling really horrible - no appetite, yet hungry, sick, nervous, tired, etc.

After we got there a bunch of the employees I know (respiratory therapists that have been to our house over the years, nursing people, etc.) came out to say hello.  And a few people said they were excited to hear our story.  Crazy!

When it was time for me to talk, Mandy walked us all up to the front of the room and introduced all of us.  She had Harlie say "hi" into the microphone and her little voice was so clear and cute - the whole crowd melted!  Good Harlie, get them all warmed up for me!

Then it was my turn.  I stood behind the podium (which was great because I didn't have to hold my papers).  I told them I woke up with a sore throat (you could definitely hear that my voice wasn't right) and said I apologize if I cough.  Then I started.  As soon as I said my name, I wanted to cry.  WTH?!  So, I said, "Sorry, it's hard to talk in front of so many people."  And after that, I was fine.
One thing I want to say before you read my speech is that it was a very "cozy-like" feeling in the room.  Everyone was sitting at big round tables (about 75 people).  And as I spoke, I felt like they were part of it.  They laughed when I wanted them to find what I said funny and they cried at other parts (I didn't necessarily want anyone to cry, although I did a little, too).  Some things are still so hard to say out loud.  And when I was telling them the Harlie-funnies, it was like I was talking to a big group of friends - they were laughing and saying things like, what?!, no way! and oh my gosh!  It was really, really great.  And I got to show some pictures on the screen, which totally makes a speech!

Anyway, here is what I said (sorry the font is all messed up because I cut and pasted, and now I can't fix it):

Hi. Hi. My name is Christy Holton and my husband and I have three children.  Murphy is 10, Harlie is 7 and Cooper is 5.  Yes, we had another child after Harlie.  Might as well get it out there that according to most of my friends and family, I’m pretty crazy.  

Anyway, Harlie was born with a handful of challenges.  I always struggle with how to tell people about her, without it being overwhelming. In brief, she has three main “things”:  

When I was 16 weeks pregnant, we learned through ultrasound about the first “thing” - there was a mass growing in her chest.  This mass was preventing normal lung tissue from developing on her right side.  To learn more about it and see what our options were, we were sent to Children’s National in DC.  

It was there, at 22 weeks along, that we learned, about the second “thing” - she had some serious heart defects.  Basically, her heart formed in a mirror image.  So everything that was supposed to be on the right, was on the left, and vice versa.  Her right ventricle was too small, and she had a large opening between her left and right ventricles.  

The combination of these heart and lung defects left us with only a 5% chance of ever bringing her home.  If she made it to delivery, she would need to have heart surgery within a few days of being born.  We waited and hoped.  We hoped that it wouldn’t be as bad as they were thinking.  And we hoped that there wouldn’t be more “things” that couldn’t be detected prenatally.

I was induced in DC on Monday, September 25th, 2006, three weeks before her due date.  As soon as she was born, I saw she didn’t have an ear on her left side.  Something definitely looked different about her face.  And she wasn’t crying.  They took her away immediately.  It wasn’t long before a doctor came in with a list of issues.  I remember the casual way in which he told us that she would need a trach to live.  She was in the OR at the time, getting intubated, because she couldn’t breathe.  I remember thinking that I had never seen a baby with a trach before.  Oh, how little I knew...

Later that day, we learned about the third “thing” - she has Goldenhar Syndrome.  It’s an asymmetrical craniofacial syndrome.  In her case, she’s missing her left ear, her left eye didn’t close properly, she had skin tags on both sides of her face, and her jaw was severely underdeveloped, which caused an upper airway obstruction.  This did not allow for breathing through her mouth and nose.  And since she couldn’t breathe, she certainly couldn’t eat by mouth, so she needed a g-tube.  It took us a few years, but we eventually learned that she was hearing impaired, even in her good ear.  

She spent her first 6 weeks in the hospital.  We learned she had some spinal defects, too.  She had her first open heart surgery when she was 4 days old.  She remained intubated (and they wouldn’t let us hold her) until she got her trach and g-tube at 16 days old.  

All total, Harlie’s had 40 surgeries so far.  I’ll just hit the highlights.

She’s had 5 heart surgeries and has a pacemaker.  After her second heart surgery, she had a chylothorax and spent more than two months in the hospital.  And she’s had 7 heart caths. This is her at six months, after her second heart surgery.

When she was 4, she had spinal fusion surgery and spent weeks in a body cast.   The bone graft they inserted in her back, died and caused an infection.  She had a wound vac and underwent 4 debridements in 7 days.  It was one of “our” worst recoveries ever.  

In our quest to give her a better airway - one that does not require a trach, she has had 4 jaw reconstructions.  The first two involved craniotomies and they harvested bone from her skull to put in her jaw.  Both recoveries required her jaw to be wired shut for 10 weeks post-op.  And in the second one the bone graft died, and left her with an infection that put her into cardiac arrest in the OR.  Clearly, they were unsuccessful for decannulation.  

Not ready to give up, we did more research and when she was five, we took her to Boston Children’s Hospital, where they harvested her fibula bone from her lower leg and created a jaw bone on her right side.  That went great.  And while she gained a lot of better oral function in swallowing and trying to talk, it still did not give her the airway she needed.  So, last summer, they did jaw distraction surgery.  

That’s where they essentially break the bone and put pins and rods on both sides of the breaks.  We turned the pins each day, re-breaking the bone, which caused new bone to grow.  We did that for 27 painful days.

This is the screwdriver-like tool we used to turn the pins.


And the pins are there, under her ears.

That was our most successful advancement and we believe she has a really good airway today.  

Of course, nothing is ever simple for this girl, which brings me back to the first “thing” we learned about her - her lungs.  So let me go back...

That chest mass that we found prenatally continued to grow and at 10 months old she had to have most of her right lung removed.  

This allowed her to eventually come off oxygen and she did well for many years.  Unfortunately, through a recent heart cath and CT scan, we learned that what is left of that right lung, does not have enough alveoli to produce enough oxygen for her growing body.  While the rest of her right lung grew, it either didn’t grow normally, or what grew has been damaged through years of anesthesia, pneumonia, and atelectasis, leaving her dependant on supplemental oxygen for the foreseeable future.  

It seems that the best way to deliver the oxygen is through her trach.  So, after all we’ve done and all she’s been through, decannulation is off the table.  For now, anyway.  

I think it’s safe to say that her lungs are not going to carry her as far as we would like.  And our next step is to see what we can do to give her the best life possible for as long as possible.  

I know that sounds like a lot.  And it is.  But, I can tell you, without a doubt, that she is a very happy little girl. 

And clearly, she knows how to relax.

She is very loved and she knows it.  She now wears two different kinds of hearing aids and is hearing very well, which has really expanded her vocabulary.  And every day she says more and more things that we can understand.  She signs and she’s learning to read and write.  She can count by 10s and 5s and she’s working on learning how to count money.  Overall, she is really doing great.  Which is amazing when you think of all she’s been through.  

When I think of the past seven and a half years, I don’t know how we’ve all survived - Harlie included.  I can tell you for a fact, that we couldn’t have done it without our home health nurses.  

I remember being in the hospital before we brought Harlie home.  They told us that we were going to need home health nursing.  I was terrified!  I actually said, no thanks!  There was no way I was going to let some stranger in our home for any kind of child care.  Every shaken baby story seems to be about a nanny in a private home.  No thank you!  

We brought her home November 2nd.  I think I lasted less than a month.  She was 24-hour care, no doubt.  And my husband and I quickly realized we couldn’t keep up.  After almost fatal trial and error, we learned that she could not sleep in her room upstairs.  So, we moved her downstairs, into our living room.  And we slept on the couch.  We have a sectional, and he would sleep on one end, and me on the other.  In thinking about those many months, I don’t know how we did it.  It was harder than I could ever imagine or ever describe.  

By December, I didn’t know how I was going to handle getting through Christmas.  We had a two-year old son, after all, skipping it wasn’t an option.  So, I broke down and called some nursing agencies, and one found a nurse for us.  Leaving Harlie with her was so scary.  And after only a few days, it was clear she was not the nurse for us.  Harlie was on continuous feeding then.  And when I returned home from Christmas shopping I found out she had not fed her the entire time I was gone. (here, I could hear, what? and oh my gosh! from the audience) I thought we were just going to have to go it alone and make it work, somehow.  

During one of our first visits to the pediatrician, Harlie’s trach was a mess.  So, the doctor called her nurse in and she was like ______ and voila!  (the blank is where I made a hand motion to describe her "magic" of working with her trach) I said, whoa!  How’d you do that? She told me that she used to be a home health nurse for a girl with a trach.  I lit up when she told me that.  I had known this nurse for years - since this was my son’s pediatrician, too - so she wasn’t a stranger!  I begged her to come work with us.  Every time we were there (which was a lot) I would ask her again.  After I told her about what happened with that nurse, she finally gave in.  I guess she felt sorry for us.  Her first night was Christmas night, 2006.  And seven and a half years later, Dawn is still one of our nurses.

She started working some nights for us.  Just two nights a week allowed us to actually sleep in our own bed and gave us the energy we needed to get through.  But, the writing was on the wall.  I needed more help than that.  Harlie was on continuous oxygen and continuous feeds.  She had terrible reflux and vomited all the time.  And she had an average of 3 to 4 doctor’s appointments per week.  So, we started looking for another nurse.  A friend of ours knew a nurse and sent her our way.  We clicked instantly.  She started in February of 2007, and Brandy is still with us today.  All total, we have had seven nurses, and we were unhappy with only two of them.  Not bad.  

And for the girl who said “no thanks” to home health care nursing, I cannot imagine living without them.  

Our nurses allow us to try and live as much of a normal life as we can.  Tom and I go on as many date nights as possible.  During the day, they have become my partners in managing Harlie’s health care.  They help me come up with feeding and medicine schedules.  They help me figure out what’s wrong, when I should stop and pay more attention to an ailment.  They help me with ordering her supplies and calling in prescription refills.  They allow me to focus on a conversation with a doctor during an appointment.  They allow me to be a mom to my other children.  They allow me to have a life.  A life where I can have friends, go to the gym and run.  Tom is a cyclist and I am currently training for my second marathon.  I might have mentioned earlier that I’ve been told I’m pretty crazy.  

You would think that with experience, I would get better at handling this life we’ve been given.  But, I still find it challenging to manage.  I forget a lot of things and I’m easily distracted.  We’ve been ordering supplies almost every month for the past seven and a half years.  You’d think I’d have it down pat.  But, I don’t.  It will be Friday (which is our delivery day) and I’ll realize we never ordered more oxygen tanks.  

Our house is a crazy place, one of my favorite respiratory therapists has called it “organized chaos.”  I think the “organized” is a bit of a stretch, but I certainly appreciate the encouragement.  Cooper will be starting Kindergarten in the fall, so I’m hoping things will calm down at home while he’s there.  

As hard as the last seven and half years have been, they’ve also been wonderful.  While we have experienced the lowest of lows, we get the perks of experiencing the highest of highs.  Literally, every day, Harlie amazes us with something she says, or does.  Noteable recent events are when she bolus fed herself a can of pediasure.  And just a few days ago, after being without nursing for a few days in a row, I jokingly said, “Harlie, you need to be suctioned, go do it.”  And she did!  She didn’t wash her hands first, and she touched the catheter like nobody’s business, but she did it.  And she was so proud!  

She’s a smart, funny, sweet girl, who complains so little.  And our nurses love her and she loves them.  They have become part of our family.  And they have been an integral part of helping her live the best life possible.  

Here, I ad libbed a bit. I looked up and said something like, "I don't know what all of you do for Pediatric Connection, but I can tell you that I feel like you are all on my team. If what you do helps someone else do their job better, which ultimately makes our life better, it matters.

So, thank you.  Thank you for all you do to help us live this life.  

For being understanding when I forget something or can’t remember what something is called.  

For handling the paperwork, so I don’t have to see it.  Or file it.  Or do anything with it.  

For delivering our supplies and packing the boxes accurately so I get everything I need.  

For checking our equipment and making sure that the machines are working right and for replacing them, when they aren’t.  

For coming to our house at all hours of the night because her heater won’t stop alarming or she needs a bigger oxygen concentrator.

For recruiting nurses to help families like mine, and for treating our current ones well so they stay happy while working here.  

So many things you do makes our life better, even if you don’t always know it.  And that’s a really big deal to me and to Harlie.  

Thank you so much.

And they all started clapping and gave me a standing ovation!  I was so moved!  I was trying so hard not to cry.  I think I was up there for about 15 minutes, give or take, and I didn't cough ONCE!  Wow!  

Then Mandy took the microphone and told everyone that they wanted to give Harlie a gift.  The last time she was in the office she loved their giant giraffe.. here is the picture from that day...

So they gave her a huge stuffed giraffe, which was so, so sweet!  And they gave me a t-shirt.  It was a great experience, really.

After I left the room, they breaked for a few minutes and a bunch of people came out to give me a hug or meet me.  It was so awesome.  The owner said, "You could hear a pin drop in there, they were hanging on your every word!"  I heard a lot of thank yous and such.  It was so great.

And I felt SO much better.  On the way home, Jamie told me that Harlie accidentally hit her toy she was holding (our neighbor, Adam, gave Cooper a large-ish tow truck that she has claimed as her own and she wanted to take it with her, so I let her).  Anyway, the truck made some noises and Jamie said that Harlie was like, oops!  and tried to find the speaker part to cover it to quiet it.  What an amazing thing when a child can see that the room is quiet and she should be, too.  Seriously.  I am so darn proud of her!

I should have gotten some pictures, but I didn't think about it at the time.  Darn it.

Anyway, that was it.

Now, today is the last day of school for Murphy.  Harlie's "school" will continue.  She is enrolled in summer school and her teacher will continue to come here for that.  And I have arranged for more teachers to come this summer, too.  She will technically be in second grade next year.  But, I feel like the lines are getting all fuzzy and it's pretty hard to think of her academic future.  As I see all the fun pictures of kids enjoying the end of school on Facebook, it definitely makes me sad that she misses out on so many of those normal experiences.  But I have to stay focused on the fact that she is doing great and maturing and saying something new every day.

Just the other day, we were walking the dog and she pointed to the sky and said and signed something.  Of course my mind went to all the normal things you might see in the sky.  And whatever she was saying and signing didn't match.  I finally had to stop the stroller and I asked her to sign it again.  I had to disconnect my mind from her pointing to the sky and focus on the sign alone.  She was signing "elephant."  Ahhh... so I said, "Harlie, are you telling me that cloud looks like an elephant?"  She said, "yes."

How many things does she think, but can't get out of that mind of hers?  And how many things do I not understand?

And just a second ago, Jamie came in to ask me how many times I've exclaimed, "That's it!" when I'm upset with the kids.  I answered, "thousands, of course."  And she told me that Harlie kept on telling Cooper to "stop it!" when she finally said, "That's it!"

I enjoy every single moment of these new verbal outbursts.  Every. Single. One.

Happy Summer Friends! And God Speed.  I'm not sure how we're going to survive, but I'm pretty sure we will.

Christy xo