Stoma Closure Soon!

Harlie is doing great.  I took her to her 9-year old well check with her pediatrician yesterday.  And I think it was the shortest well check appointment she's ever had.  There was nothing to discuss, really. I can't tell you how great that feels!  She had to get a flu shot.  She can't get the mist because of her medical issues.  And when she realized she was getting a shot, she lost it.  That girl is a fighter, through and through.  And she is done with getting poked, I can tell you that.  It was not a fun experience for any of us.  It's not the shot that gets her, it's the anxiety and fear of what's going on that does.  It looked like an episode of PTSD, to me.   Poor kiddo. For as happy as she is, she still has too many bad experiences and memories to go with them.

She now weighs 48 pounds and is 46" tall.  She is not on the chart for height.  

But, health wise, she's doing great!  We went to DC to see her ENT on September 9th.  Here we had been thinking that her stoma (trach opening) was closing nicely, and the first thing her doc said was, "Wow, that hasn't closed up at all. I bet I could get a trach back in."  Please don't, I thought.  I had to laugh.  I guess you see what you want to see, so we thought it was tiny.  Oh well.  

The good thing is that he said he needs to close it.  I know in some cases they wait a while.  But, in hers, it is a pain in the butt.  It leaks really bad and it makes an annoying sound most of the time.  It rattles because of the secretions.  Plus, it really reduces the clarity in her speech.  He said his OR schedule was about two months out.  But, when they called me to schedule it, he had an opening on October 9th!  Woohoo!  That's a Friday, and she will stay in the ICU at least one night. 

Tom and I are signed up to do the Rugged Maniac on the 10th, but stoma closure is way more important.  Plus, I don't like to get dirty when I run.  So, to be honest, I wasn't that upset I would have to miss it.  

This will be Harlie's first time going under anesthesia without her trach.  And her mouth doesn't open very much (it's hard to get a spoon with yogurt in her mouth, it's that small).  So, she cannot be intubated as normal.  So, they are going to have to try some other ways.  As far as the surgery goes, he doesn't just stitch up the hole. He has to actually remove all the scar tissue that's there, so the fresh wound will heal on itself, basically... I think that's what he said.  And he said that she will come out of the OR with a small trach in.  We will wait for her to wake up and then we'll decannulate (take the trach out) again.  I told him I was so glad he told me that.  I would have been really upset when I saw her come out with a trach!  

Anyway, we've been counting down the days.  Then yesterday, I got a voice mail from our nursing agency that said our Medicaid waiver (Harlie went on a different waiver after the trach came out) put us on an Optima insurance policy.  I listened to it as we were rushing out the door to go to her well check appointment.  And I thought, okay, whatever, as long as she has Medicaid, those are details I don't care about.  Moving on...

Then, a few hours later, I got a call from Harlie's ENT office.  She said that they were working on the insurance approvals for Harlie's stoma surgery and that the hospital doesn't have a contract with Optima!  WHAT?!  Yeah, that's a problem.

So, in a panic, I start making some calls.  Apparently, we were just put on this Optima plan as of October 1st.  I HATE paperwork, and I get a lot of it.  So, I dug through my pile, and found a letter dated September 23.  It doesn't sound urgent at all. But, it says that we are going to be put on an Optima plan as of October 1st, and if we would rather be put on a different plan (it included a brochure for several different plans) we had to notify them by September 30.  Hmmm, so they printed the letter last Wednesday.  Maybe it went out in the mail that day, maybe the next.  I might have gotten it by Friday, which might have given me two business days to make a change.  I'm sorry, but I do not look at all my paperwork in that kind of time frame.  Nor have the time to call all of her doctors/hospitals to see what plans they accept.  So, crap.

In the brochure, each plan (Anthem, Optima, etc.) lists the facilities that contract with them.  There are four plans and not one of them contracts with Children's National Medical Center in DC.  It is the only major children's hospital around here.  So, I asked her which plan contracts with CNMC and she said, "It's not in Virginia, so none."  Now, I get that it's technically not in Virginia since it is in the District of Columbia.  But, seriously???  I can tell you for a fact that CNMC sees plenty of Virginia Medicaid patients.  What the hell?!  I called Harlie's ENT office and asked her if they take Anthem, and they do.  So, that's weird.  Clearly, they don't want the average Medicaid recipient to know they can, in fact, go to CNMC.  Damn, it's hard to need specialized services - in EVERY area (medicine, education, etc.)  It feels like the services are there, you just have to work hard to find them.

So, I explain my situation, and shockingly, she appears to be sympathetic.  She told me who to call and after a few more phone calls, I get the right person on the phone.  I'm thinking that I can switch to Anthem.  But, that switch won't be effective until November 1st, which doesn't help me on October 9th.  So, the nurse case manager with Optima put in an "urgent" request for review for an out of network approval.

I don't want to move this surgery back.  Not even one day, much less however long it would take for me to reschedule it.  We are going camping in a few weeks and the thought of trying to keep Harlie's stoma clean for three days in the woods, around an open fire, with no running water, sounds downright impossible.  It would be awesome if it could be closed, and all healed by then.  Now I just have to cross my fingers that it gets reviewed, and approved before Friday.

Plus, Harlie is ready to go back to school.  But, I am not sending her until that stoma is closed. I have realized that Harlie gets more stares with a hole in her neck than she did with a trach in her neck.  A few weeks ago, we went to Kings Dominion for the Altria Friends and Family day (my niece works there and gave us her free tickets).  I want to post more about that day soon.  For some reason, I found myself surprised with the staring.  Harlie was on a train ride in the kid area and a little girl was in the car right in front of Harlie.  So, Harlie was the first one in our car, and the girl was the last one in her car, putting them close together.  That little girl stared at Harlie the entire time.  I know people have to gather information, and that most people have never seen someone with a hole in their neck, but come on.  Even a parent couldn't look away when we were standing in line.  I get it to a certain degree, but I'd much rather them just ask me what's going on.  I'm always happy to share and educate others about Harlie.

Anyway, since Harlie wants to go to school for the first time, like ever, and she's currently super proud of her naked neck, I don't want her to go and get negative feedback from other students.  I feel like we're in a delicate mode of progression regarding her education, and I don't want anything to disrupt that.

Speaking of... I can update you on her education, too.

On September 24, we had a component meeting for her upcoming IEP meeting.  Last year, while on home bound, Harlie's teacher came to our house during the morning/early afternoon.  And that worked out great.  The boys were at school, the house was quiet.  And elementary aged students have proven to do better learning in the am.  So, all was good.

This year, her teacher is not allowed to come during the school day. This is another area where it is a struggle to get the specialized services you need.  I don't understand the way it's set up or why this isn't allowed, so it makes it very difficult for me to solve the problem.  So, she now has two different teachers that come to the house, AFTER school hours.  This has proven to be very challenging and not nearly as effective.  The boys are home from school, and two days a week, I have to rush Cooper to swim lessons at NOVA.  Since I have to take Harlie with me (it's after Brandy leaves for the day) and we don't get home until 3:30, her school day doesn't start until after that!  A kid with no barriers in learning would have a tough time learning under those conditions!

So, during the meeting, I gave the team an update on her medical situation and explained that I would like to try to send her back to school after stoma closure/recovery.  There are some challenges, though:

1.  Her endurance.  So, we are going to start with half days first.  And go from there.  We'll add more time/classes as she can tolerate.

2.  Potential sickness.  She was sick all the time when she was in school last.  So, I want a plan in place for her to quickly get services at home again, should she need them.

3.  Nursing.  As of right now, I don't have nursing coverage Monday through Friday.  So, I don't know that I can even send her every day.  So, I would like to be able to send her when I can, and on the days I can't, I want them to come to the house - during the school day.

4.  School hours.  Until I can send her back to school, I want to change her school hours from after school, to during normal school hours.

5.  Grade level.  She is technically in 3rd grade this year.  But, developmentally, she is not as far along as typical 3rd graders.  She is really struggling to learn how to read and all of her education is on a 1st and 2nd grade level.  So, I don't think it makes any sense to put her in 3rd grade.  She won't be able to spend any time in her class anyway.  She'd have to be pulled out for all of her learning.  So, what's the point?  And making friends would be almost impossible, considering they wouldn't be together during the day.  My argument (and desire) would be to put her in 2nd grade, again.

So, they are working on those things and hopefully, all will work out the way I want, which I think would be the best for Harlie.

She really is making a lot of progress, overall.  We used to have to make her respond to a "Hi Harlie" greeting by saying, "Hi" back. Every time a student would greet her, she needing prompting/instruction to respond or wave back.  This went on for YEARS.  Well, on Wednesday, I took her into Cooper's school to pick him up to go to swim.  While we were waiting for him a little boy came into the office.  She said hi to him - unprompted.  Then as we were leaving, she waved to a little girl in the hallway and then said, "Mama, I waved to her."  That girl seriously cracks me up.  She knows darn well that we've been working on that for years and she wanted me to know that it finally paid off.

I think she's really proud of herself.  Which is also why I don't want to put her in a class where she knows she doesn't belong.  She's smart enough to know that she's not on the same level and I think it would be detrimental and never ending.  Every year would be the same.  I think she has a way better chance of "catching up" if they put her in 2nd grade this year.  I know schools have a problem retaining kids more than once.  Typically kids get too big for the grade.  But, as luck would have it, that's not an issue for Harlie.  She is the size of a first grader, so she will still be on the small side of a 2nd grade class.

So, we'll see what happens.  My fingers are crossed that her team, and whoever is higher up in the chain that has to approve of going ousidet of the box, see that my requests are reasonable and in Harlie's best interest.

Well, that's it for this post.  Hopefully I'll have another post soon.

Thanks for reading!
~Christy xo

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.

Ugh.

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

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."

Crap.

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.

Thanks!
Christy xo

Harlie's first day of school...

My posts are going to be out of order.  But, it's the only way I can post right now.

Over the weekend, Harlie's Vest arrived.  A nurse called me to make sure everything was in order and to schedule our training time.  On Sunday, she came over to train us.  It took over an hour.

We have to do it for twenty minutes, two times a day.  Harlie isn't a fan.  The vest inflates and then air pulses through the tubes into the vest.  It's purpose is to help move mucus from her lungs.  Hers is ordered for use all the time - not just when she's sick.  I guess if she doesn't get sick much this winter, then that means it's working.

Last night was our first time doing it on our own.  We put it on Murphy and Cooper first, just so Harlie would think it was "fun."  But, she's smarter than that.  She knows it's not fun.  I was a little nervous about this morning's routine, with the Vest added into it, since it was going to be Harlie's first day of school!  The problem is that the Vest can't be done near a tube feeding time - which was 6am. So, I had Tom give her the first half of the can at 5:30 when he left for the gym.  Then I got up at 6am to give her the rest of the can and her meds.  Then I let her rest a little while longer before I got her up and dressed.  We started the Vest treatment a little ahead of schedule, before 7am.  She gets her breathing treatment at the same time.
 

Not a fan of the 20-minute treatment.
Bribed her with the iPad.

This bad boy is NOT cheap. (like $16,000!)
It's also not light.  And we will have to take it with us
when we travel to Boston, or anywhere else.  

Of course my healthy kid asks if he can have a turn next.
And then she kicked him. Sibling love...

Despite her initial reaction when told it was a school day,
I think she was probably a little happy to be going.

We travel light.

She's going to have to use her chair for a while.  Her classroom is SO far from the parking lot!  There is no way she can walk that far in a reasonable amount of time.  Especially with her oxygen requirement.  And her questionable spinal pain.  I'm rationalizing that it will mean for more energy for her work.  But, secretly, deep down inside, it kills me that she needs it.  I never thought we'd be here when she was this age (almost 7!).

Since I didn't get to go to open house at her school, I wanted to go in the class and meet her teacher.  As we got to her classroom, the class across the hall saw her and they all said excitedly, "Harlie's here!"  I had to really choke back the tears.  Just thinking about it now makes me cry.  They all seemed genuinely happy to see her.  Her teacher said that every day they would ask her about Harlie.  I love how kids are so concerned about her and how she's doing.

In class and somewhat cooperative.

Terri said she did all her work.  She just did it at her own pace.  Which is slooooow.

Last week her teacher sent home this banner the kids made.  The kids signed their names on a heart and glued it to the banner (and her teachers, too).  It is very big and takes up most of my main wall in my office.  I love it!

And I've been meaning to show you this puzzle a group of girls made for Harlie.  It is one of those really big floor puzzles.  A friend of mine has a summer camp for girls and they did this puzzle and made some other cute stuff for Harlie.  I love all the sayings on it - Never give up, Be yourself, Just keep swimming, She leaves a little sparkle everywhere she goes, Don't worry be happy, Be unique, Spread love, etc.  So cute!

In preparation of Harlie going to school today, I made a few checklists to make sure we had everything we needed.  I got a little chuckle out of her backpack checklist.  Notice the last item... oh, yeah, that school book.

We're going to need a bigger backpack.

Seriously, when I picked her up today (half days until she builds up a little endurance) I had to carry her school stuff in my hands.  We really do need a bigger backpack.  The one she has is a kid backpack (large, but designed for a kid to wear).  But, considering we are the ones carrying it, we need to get an adult backpack this time.

I have more to write and more pictures to share, but will have to do it tomorrow.  Thank you for all the love!

~Christy xo

Back in the hospital

Whew!  I can't seem to keep this blog updated.  Harlie is a moving target lately.

Monday was Labor Day.  Tom and I took the boys to the pool.  Harlie can't go because of the PICC line (which can't get wet) and of course, the insane amount of oxygen she's on.

Cooper, Philip (our neighbor) and Murphy

Tom throwing Cooper.

Murphy, Philip, Kaden and Cooper.

I took the last photo and posted it on Facebook.  After seeing so many happy pictures and status updates about how great summer was and how sad most people were that it was over, it made me think about how I felt about this summer.  My caption - Good effin' riddance Summer 2013!  I wish I could have hash tagged it (which I never do) as "worst summer ever" but - sigh - it's just too close to call a clear winner.  There was that one summer that I feared her death the whole season... so that sucked, too.

Anyway, I'm not sure where our current situation falls.  The end of summer or the beginning of fall?  Because if it's the beginning of fall, that's not a good sign.

Tuesday, September 3

The first day of school!  Murphy rushed out the house to go get Philip, so I missed our photo of him under our tree.  Darn it.  So, Philip's mom and I followed them there.  I carried Murphy's school supplies (since I forgot them the other night) and took photos.

Philip and Murphy

Philip and Murphy.  I don't know why Murphy
has such a hard time with photos... grrrr.

Crazy.  

Murphy unpacking.  

Murphy ignoring my request for a smile.
Looks like he wanted to get serious about school.

Of course the bus came to the house for Harlie.  When they tried to reach me last week, I was in the hospital, so we never spoke.  The bus came and Tom had to go out there to tell the driver she would not be taking the bus for a while.  It made us both very sad.  To be surrounded by healthy children and know that one of yours isn't, is hard.  Living with grief every day....  And I've been feeling a lot more anger than usual in the past eight months.  I just feel like I'm running low on grace.  We have such an extreme situation in so many ways.  She's not textbook anything.  One problem complicates another problem's solution, etc.  And when things are bad for a while, I always start to wonder, is this the beginning of the end?  Is this going to be our new normal?  I hope not.

I spent the morning making a bunch of phone calls and sending emails.  And I scheduled an appointment with Harlie's pediatrician for 1pm.   I sent Cooper to Bethany's house, and Terri and I took Harlie.  As we were walking out the door, she realized where we were going, and started to cry.  She really has so little energy and had no interest in getting off the couch.  Then she signed "potty."  Never fails.  She cried and sat on the potty for a little while.  It occurred to me that she could have been stalling.  So, I told her that Dr. Derco was not going to hurt her.  And she got off the potty and went into the car.

Her doctor said that she had pitting edema, which basically means that fluid was accumulating in her soft tissues.  I guess her lungs were running out of room.  She's also up four pounds from just three weeks ago.  Anyway, he said she needed more Lasix (a diuretic to help her body move the fluid into her kidneys so she could pee it out).  Unfortunately, her one daily dose was scheduled for 2:30, so it was going to be a while before we would be able to see a difference from adding a dose.  He called her cardiologist and then her cardiologist called me a little while later.  After her doc was done with his exam, I said, "See?  No hurt.  Remember when I told you that it wouldn't hurt?"  I really want to get her to the place where she trusts what we say.  What a difference that would make with her fears (and subsequent behaviors)!

We rushed home after the appointment because I really wanted to walk with Murphy home so he could tell me about his day.  At about the time I was leaving to get him, Harlie's Medicaid case worker got to my house.  She did an overall assessment of our situation and upped our nursing hours from 10 a day, to 16 a day.  So, that's good.  Luckily, the school was running late (first day and all) so I made it in time to chat with a few parents and then walk home with Murphy.

Classmates after school.

He had a great day and likes his teacher, so that's awesome.

Then we went home and I spoke to Harlie's cardiologist.  He wanted her to have IV Lasix instead of oral Lasix.  So, I called the infusion company to see if they could provide the IV Lasix.  The manager that I spoke with said it wouldn't be until Thursday till he could get it.  And if she needs it, she can't wait that long.  He also said that he wouldn't even be comfortable with us giving it to her at home.  It lowers blood pressure, so he would rather her be monitored.  I think the general thought was that if she needed it, she needed to be in the hospital.  But, we are not the norm, and I have IV access (clearly not the norm) and I have a mini-ICU at my house.  Maybe that makes me feel like I'm more capable than I really am to keep her home and nurse her through this.  I don't know.

Yet I still made her come with us on a walk in the evening with the dog.  She didn't want to go, but we put her in the jogging stroller with an oxygen tank and I think she enjoyed the fresh air.  It's always so fun to see Rooney run his heart out!

Anyway, come bedtime I gave her a second dose of Lasix.  After that she only peed once.  Whereas the last time I gave her a second dose at bedtime (Saturday night) she peed twice.  The night was rough and I had to get up several times to see why she was alarming.  Now that she's on so much oxygen, if she alarms, I have to go in immediately to see why.  Her tubing could be disconnected or something and without the oxygen, her sats sink in to the 60s in a matter of seconds.  No exaggeration.  Anyway, I was tired this morning for sure!

Wednesday, September 4

Once I was up and Murphy left for school, I emailed her cardiologist and pulmonologist to give them an update (and to tell her cardiologist that we couldn't get the IV Lasix).  Dr. G. (cardiologist) emailed right back and after going back and forth a few times with more details, he said it would be best if I just brought her into the ER now.  Her belly is distended and that might mean that oral Lasix wouldn't work as well, so she needs IV Lasix.

I was walking the dog with Cooper when I got that last email and was kinda far from the house.  So, it took me a while to get back.  But, that gave me some time to get Cooper squared away.  My friend Michelle had just sent me a text telling me she was available, so I called her.  Her and Sally came right over.  They took Cooper to the park.

Then Cooper and Rooney went to Bethany's house for the rest of the day.  And Murphy went there after school.  Then the boys went across the street to Philip's house for dinner.  His mom is making dinner for them tonight and tomorrow night.  Oh, what would I do without my neighborhood???  Even though our life is kinda crazy, they are entertained, loved and well taken care of.  So, thankful!!!

As we pulled into the parking lot of the hospital, Harlie saw where we were and yelled, "No!" from the backseat.  Ugh.

So, we got to the ER and saw some old friends.  A nurse that we had years ago was back!  It was so good to see her!  She went to a different department for four years and today was her second day back in the pediatric ER.

Then Dr. G. (her cardiologist) came by to see us.  And they did an echo (ultrasound of her heart) just to make sure all the extra fluid wasn't negatively affecting her heart.  Then they got a chest x-ray and an abdomen x-ray.  Her chest x-ray definitely shows a lot of fluid - on her right side especially.  And it's now in the chest cavity instead of just being in her lung tissue.

She's now been admitted into the PICU (last time we were in the progressive care unit, which is a step down from the ICU).  Funny how you get comfortable somewhere.  At first I hated the PPCU, but being in that room made it so much better.  And I got to know the nurses and team.  In the PICU, it's a whole different team.  Of course, I'll get used to it here, too.

She's also doing a lot better than when we got here.  And she immediately asked to sit in the chair, with the table and the computer.  Of course, she's not been fed or had any water flushes since 10am.  And apparently, you can be dry in some ways and fluid over-loaded at the same time.  So, that makes getting her balanced easy.  Not.

They have now started her on an IV Lasix drip.  Her blood pressures have been low today, so they had to get some meds to have on hand just in case they need to give her something quickly to raise them.

We already had some visitors - Niki and Katherine - some of my running friends.  I forgot to get a picture.  Darn it.

It is now after 9pm.  And I am pooped.  We are about to give Harlie a bath, and put her to bed.  Then I think I'll go home for the night and return early tomorrow.

Oh, one other thing... today at Harlie's school, a lot of the moms organized all the kids who had We heart Harlie t-shirts to wear them today to school.  So sweet!

This morning.

Thank you for all the wonderful support and love!!

Much love,
Christy xo