General Update

Hi!  I'm sorry it has been so long since my last update. It has been a busy time.  Here's a snapshot of what has happened in the last few months:

September
Murphy's first season of high school cross country.
Harlie turned 12.
Cooper turned 10.
We Heart Harlie and Friends - first Gala.

October
Family camping trip.
Harlie's appointment with her orthopedic doctor for her spine.
My trip to Sedona, Arizona to meet up with friends/moms of complicated kiddos.
Halloween

November
Chris Stapleton concert.
Harlie's eye appointment (since she "failed" the visual screening at school).
My mom's spinal fusion surgery.
Local ENT appointment.
1st West End Orthodontics Turkey Trot benefiting We Heart Harlie and Friends.
Thanksgiving.
Harlie's scope of her airway and lungs.

December
Mumford and Sons concert.
Steelers vs. Patriots game.
Harlie's trach scar/wound issue.
My birthday.
Christmas.

So, where to start? I guess I'll start with her scopes on December 1st.

She had a CT scan of her lungs, then went into the OR for a scope of her airway by her ENT, and then a scope of her lungs by her pulmonologist. All of this was technically supposed to happen months ago.  The last time she was scoped was in May.  They wanted to do it again during the summer/fall to see how things were looking and to see if there were any more signs of Plastic Bronchitis (the last sign of PB was in February 2018).

This all was scheduled for October, but she got sick right before, so we had to cancel.  Then the next proposed date was at Halloween and since that is her favorite holiday, there was no way I was going to risk her missing it.  So, we were still working on finding another date when both of her doctors were available.

Then, on Thursday, November the 8th, I noticed her scar around her trach stoma looked odd.  I took a closer look and realized that the scar tissue looked puffy, like it was full of fluid.  Her stoma had such a hard time healing after her airway reconstruction that it was hard not to freak out and be so fearful that it was going to open up again.  That would be SUCH a disaster.  And she's had a healed wound/scar tissue dehis (open up) before that almost ended very badly, so the fear is real.  That was eight years ago and I remember it like it was yesterday.

Anyway, I sent some pictures to her ENT in DC and her local ENT and they called in some Clindamycin. It did seem to help, but by day 11 of the antibiotic, it was still puffy.  So, I was scared that it was going to get worse again as soon as the Clinda was stopped.  So, we saw the local ENT.  She suspected that it was filling up with fluid and/or air.  So, she was thinking it should be drained while she was under anesthesia.  Since we had this scope that still needed to be done (also under anesthesia) her ENT in DC went on ahead and got it scheduled pretty quickly.  Of course, within just a few days  of seeing her local ENT, the scar tissue settled down and did not fill up with fluid and/or air again.  Typical.  But, we continued on with the scopes anyway since they needed to be done.

As far as how things went... it is kinda hard to explain. It is hard for me to understand, and even more difficult for me to help others to understand.  But, basically this is how I understood it:

Airway
The horrible scarring you see on the outside of her stoma, also exists on the inside of her trachea.  Scar tissue in your airway is not good.  He said that it basically healed around her trach cannula (the tube that goes in her airway) so it formed like a sleeve.  I asked if he could remove it, but it is not that simple.  So, no, he cannot. He might be able to do something in the future but, that is too far away for me to think about. Anyway, her airway is so friable, irritable and angry.  He said the slightest touch makes it bleed. He said it is almost as if her body doesn't like the trach.  And as he was saying that, he said maybe her body is reacting to the material the trach is made out of.  She had this brand of trachs for nine years.  Of course it makes sense that her body wouldn't like that material now. Ugh. So, he said that if it doesn't calm down in time, we could try a metal trach.  I know adults use them, but it sounds uncomfortable to me. So, we'll see.

In summary, we can safely and easily do trach changes, she has a voice, and her airway is stable.  So, we are going to leave it alone for now.  She has some things coming up that require her to go under anesthesia, so there is no reason to even talk about trying to get the trach out. Which, honestly, I think is a pipe dream at this time.  There are so many obstacles in her path.  It makes me more sad than words could ever say...  Her airway was GREAT on August 7, 2017....

Lungs
The good news is that her lungs in general, looked good.  He saw no evidence of Plastic Bronchitis, which is great and a huge relief.  However, the CT scan showed some malacia (floppiness) in the lower left lung.  This is a new finding. And might explain why she is so easily winded and out of breath.  Since she had almost two of her three right lung lobes removed, she NEEDS every inch of her good lungs.  When ANY part of her good lungs are compromised, she feels it.  She has NO reserve, and never has.  So, it looks like she's using even less of her lungs than she had.  Not sure what this means - will it correct itself?  Will it get worse?  What caused it? I have no idea.

The past 16 months have really taxed my brain.  I've noticed that I need more time to process the information I've been given. So, I have a hard time asking all the right questions while I have the doctor in front of me.  I will have to email him later for more clarification.  He said the same as her ENT, let's leave her alone for now and give her time to heal.

So, in general, not great news.

I was feeling crummy and tired, in general.  We left the hospital in April, went back for a one night stay for a scope in May, so we had a "good" break of many months.  Yet, it felt like we were JUST there!  Eating meals while in the hospital can be depressing.  Which is why I was so thankful that my friend Mona texted me and asked if I wanted to get out for dinner.  Oh, thank goodness for good friends!  We went to my old favorite, Brookland's Finest.

The next morning, the doctor who has done almost all of her heart caths came in to see us.  He said he had talked with her cardiologist and they want us to come back in 6-8 weeks for another heart cath.  He will do a study with oxygen and then a study with nitric oxide to see how her body reacts.  If her body reacts well to the nitric oxide, then they will start her on Sildenafil (a medication that can help lower pulmonary pressures, I think).  Her pressures are pretty high.  So, so much for leaving her alone.

But, how can I say no to this? IF her body reacts well to the study, and they put her on this medication, then MAYBE her pressures will go down and MAYBE she will have higher sats and more energy??? I have no idea, really.  Another email I need to send...

So, that's that.  Not great, all around, in my opinion.

And she's getting so much more aware and curious.  When she is dealing with something painful (like her bone anchored hearing aid site oozing) she asks, "Why does this keep happening to me"?  Then, she asks if this happened to me when I was a kid.  Which, of course, none of the crap that she has to deal with has ever happened to me - or most kids for that matter.  While in the hospital she asked that several times.  It broke my heart.  I wish I could stop this from happening to her, but I can't.

She's also looking for some control.  On Saturday morning, I asked her if she needed to use the potty.  She said, no thanks! I waited a while, then asked her again. After a few hours, I didn't ask, I told her to go potty. She said, "I will go potty AFTER you take this thing out of my hand."  She was talking about her IV.  So, it was well after noon when her nurse removed her IV.  THEN, she said she would go to the potty.  So, that's how it's going to be...

She is so funny, though.  She likes to talk to everyone that enters her room.  A woman came in to change the trash bags and Harlie said, "Excuse me, what's your name?" Then she asked what her favorite color was and if she had any pets.  Haha!  Doctor, nurse, custodian - doesn't matter to her! That lady was so tickled that Harlie wanted to learn all about her.  I hope it made her day.

I wrote this post weeks ago and never got back to finish it.  It has been 7 weeks since her scope and I have not scheduled that heart cath that they wanted to do in 6-8 weeks.  Oops.  I don't think either one of us is ready to go back and do that yet.  I know that may be hard for some to understand.  But, it is true.  She is doing really well right now and I just don't want to take her back there yet.

Anyway, I'll stop here for now.  I have another post I want to work on.

Thanks for reading!  Much love,
Christy xo