Tuesday, May 19, 2020

Hardware Removal Day

I'll talk about pre-op day first, then I'll blog about today...

Yesterday we did the whole drive through testing thing.  That was really easy.  And I saw no reason why it couldn't be handled without the car, but whatever.  We let Harlie stay in her pjs since we had to be there pretty early, and she didn't have to get out of the car.  This should show how close I am to losing it, because I am NOT a wearing-pjs-as-clothes kind of person.  I firmly believe they should stay in the home.  Pajama day at school is a difficult day for me.  

Anyway, the whole thing from driving into the garage, parking in the 3rd spot (only one other car was there), having them swab the back of her throat for 10 seconds (which felt WAY longer) and exiting the garage - took 6 minutes.  I didn't take any pictures because I didn't want to get in trouble (this big city, pandemic environment has put us on edge for sure).  

So, since we were out and we had nothing else to do but go back to a one bedroom teeny tiny apartment, Tom drove us to some beachy area in South Boston.  We got out and walked this trail around some waterway.  Clearly, I live my life on a need to know basis - and I didn't need those details.  

It was a little chilly, so we just took one big loop around (I'm guessing it was over a mile loop - it was around that water you see in the picture) and headed back to the car.  These little things we do when we are here (like taking Harlie to the aquarium) keep her mind off the hospital and that is really good for her.  But, now there is literally NOTHING for us to do with her.  I feel so bad for her - she's done nothing wrong, but her already difficult life is now so, so much harder.

Tom stopped and got me a fancy coffee on our back to the apartment.  Then we just hung out there until our 1:30 pre-op appointment.  They called me before that to let me know that her test came back negative - so that was good.  Not that I was surprised, of course. They test all admits and I think they have less than 10 Covid patients and only one in an ICU.  So, that's good.  I would think that in most, if the kids test negative, than the parents probably are, too.  Especially in vulnerable kids.  So, I don't feel as fearful being in the hospital as I thought I would.

The pre-op appointment was "fine."  Although, if I'm being brutally honest, I am officially tired of answering the same questions over and over and over again.  I have had each person "update" her med list, just to learn in her room that they had Lasix listed PRN (as needed) instead of twice per day.  WTF.

We saw a nurse practitioner and had to answer her name, her DOB, her allergies, what procedure she's having, her meds, does she have any issues with anesthesia, any liver issues, has she fallen in the last few weeks, diabetes, my address, my phone number, who lives in the house with her, do we smoke (no), etc.  Then, we see an anesthesiologist and I have to answer all those same questions again.  It probably didn't help that I had to reorder her Sildenafil that morning, which takes about 12-15 minutes of me answering all the same questions I have had to answer every month for 15 months now.

Anyway, when done, she asked to go to the gift shop, which is obviously part of our routine.

Target is open, though.  But, way too many people to expose her to there, so no way.  And no one gets tested before going into Target.  But, this gift shop in a children's hospital, which could limit entrance to one person at a time, can't be open.  Got it.

So, we just went back to the apartment and that was that.

We woke up early this morning because we had to be at the hospital at 6am.  Harlie was up and ready for the day without us having to wake her up.  She was definitely more excited than scared, which is good.

We goofed around, talked to all her docs, etc. Dr. Resnick (her surgeon) showed me a selfie on his phone of him wearing his We Heart Harlie shirt.  And last time we were here, Dr. Vinson (her anesthesiologist showed me a selfie with hers!)

Then they came to take her back...

We went and got checked into the waiting room.  They are now assigning seats or areas for families.  We were in #22.

Honestly, this was a welcome change.  At first, I didn't like it.  It was just more change to remind us how awful things are.  But, after the ENT came in to talk to us, I was glad the "walls" were there.

I'll try to explain.

Harlie's problem since birth has been not enough jaw for her tongue base to lay down in her mouth.  This causes her tongue base to be in her throat, blocking her airway.

We worked for years to try and make her jaw bigger.  We always felt so lucky that she had no actual airway issues - meaning her airway itself was FINE, it was just blocked at the top.  So, we were able to focus on fixing that particular problem.  Several really hard surgeries and many years later, we finally did it.  And in August of 2015 she was decannulated (the trach was removed).  And life was so good.  For two years.

Then her jaw developed a side effect - and the bone fused to the base of her skull, locking her jaw into one place, preventing her from opening and closing her mouth.  The surgery to fix that (in August of 2017), made her jaw too small again, and created the same upper airway obstruction she had.  That is why she had to be emergently trached again.

Unfortunately, the first few months of trached life proved very difficult.  And in February of 2018, her ENT in DC discovered that the emergency trach was placed through her cricoid cartilage, instead of her trachea.  This was an airway problem.  And he had to do an LTR (laryngotracheal reconstruction).  He took cartilage from her ear, and tried to repair the damage.

If you've been following Harlie, you'll likely remember how difficult that two month hospitalization was on her.  She almost died. It was horrible.  And I definitely walked away with PTSD.

So, we went back to the drawing board and started working on fixing the upper airway obstruction.  Hence, this jaw distraction done in March.  This is actually step one of a two step process.  Step two is when they design custom jaw joints for her and then put them in?  Install them?  I don't really know the proper language there.  This first part gives her a downward/forward rotation and the second part will give her forward movement/forward length.

So, we didn't really expect for her to come out totally fixed, but we did expect to be much closer that that ultimate goal.  We hoped for an improvement, though, maybe in her speech, or her ability to wear a speaking valve or even a cap some (which allows you to breathe in/out of your mouth and nose only).

So, her ENT went in first and did a scope.  He said he saw two problems.

Problem #1:  This is a picture of her epiglottis.

The picture on the left was taken while she was sleeping, in a relaxed position.  No intervention, just a scope and a picture.  Her epiglottis is completely squished by her tongue base in this picture. The tongue base is to the bottom/left of the photo, the epiglottis is in the middle and the back of her throat is to the upper/right of the photo.

The picture on the right was taken after they moved her jaw up, in a forward position.  In this picture, you can actually see her epiglottis.

It should look like the one on the right ALL the time.

So, this isn't good.  This is an upper airway obstruction.  And this is what we've been trying to fix.

Problem #2:  Her airway.

These pictures are of her airway above the trach.  This is after he moved her jaw forward, and went through her epiglottis (I think is what he said).

Both pictures are the same picture of her airway.  

The picture on the left is pointing to her airway, it measures 2mm.    

The picture on the right is indicating all the scar tissue that should not be there at all.  All of that circled area should be open airway.  He said it should be closer to 8mm.  

He said she would need to have another LTR and maybe a rib graft should be used, since it is beefier.  

This is the really devastating part of it all.  

I could barely hold my tears back till he left.  And then I was glad for those walls.  

I don't like to cry.  I especially don't like to cry in public, as I might have mentioned.  

But, to have the hopes that we did, just to find out about this scar tissue, I just can't explain it.  

Clearly our first thoughts were that there was no way we could ever agree to another LTR.  We almost lost her and I cannot even think about doing that to her again.  In thinking that, we had so many bad memories come flooding back.  All of that was so unexpected, and we weren't prepared to have to think about all that again.  

Then, my next thought was do we have to start thinking about accepting that we will never fix her airway?  That she will live with her trach forever?  Do we have to give up?  Giving up feels so wrong, but making her go through more and more feels wrong, too.  

It all feels so tragic and so unfair.  The start of all this wasn't a surgery gone wrong - it was a horrible accident - that left her with disabilities we are still trying to fix.  

Then Tom said we have to focus on the now.  Today her metal will be gone and she will be happy.  And we have to focus on that for now, be grateful for that and deal with the rest later. 

I had to pull myself together, since we still had to talk to her surgeon and her anesthesiologist and I didn't want to be crying then.  

I did think that when things calm down (and we are not in the middle of a pandemic, hoping and praying life will get better one day) we will need to see Dr. Preciado, her ENT in DC, since he knows her airway so well.  Maybe (oh, that hope is so sneaky and stubborn) he would say something different. 

After a couple of hours Dr. Resnick came by and said everything from his standpoint, went well.  She lost a lot of blood.  But, all the metal came out okay and he thinks we got a lot of downward/forward growth.  We still need to think about step two.  But, there is no way we can think about that now, for several reasons.  

He said we could take a break.  But the custom joints take about four months to build, so we just have to keep that in mind as we think about timing.  

Hard to justify doing more to the upper airway if we can do nothing about the scar tissue in her airway.  But, not doing step two makes me feel like we just did step one for nothing.  So, as soon as we can/want to deal with it, we will have to see Dr. Preciado.  

In the meantime, we need to just let her be.  We need to let her heal and be as happy as she can be.  And we need to stay out of the hospital and give us all a much needed break.  

I know I mentioned it earlier, life with a medically fragile child was hard before coronavirus.  It feels impossible now.  And that is so true.  We've been doing this for 13+ years and we are out of gas.  Doing all of this during this pandemic might have broken me.  

The cafeteria is open, but there is no dining area.  No where to sit and eat.  No where for tired, stressed parents to get any kind of much needed break so we can return to our child's room a little more rejuvenated.  Everyone is wearing a mask and there are signs everywhere - so many signs telling you what you can and can't do.  There is so much fear.  And it has created an atmosphere where we are on edge all of the time.  

I cannot put into words how beat up we feel right now.  We are done.  We just want to be home.  

The next direct flight isn't until Friday.  So, if they let us take her out of here before then, we might just drive home.  I don't know.  We'll have to see how she does.  

She's now in the cardiac ICU and resting.  She did lose a lot of blood, so they gave her a blood transfusion.  She woke up for about a minute and she started to cry.  She said she hurt.  So, her nurse gave her some pain meds.  I snapped a quick picture during a re-positioning in bed.  

She looked so alert and good.  And when she tried to talk it was so different since she could move her jaw again!  It was still hard to understand her because everything is so swollen.  But, I have high hopes (ugh) that her speech could be improved.  

So, we are doing okay.  I feel like sometimes I'm not as honest with you about how difficult things are because I'm afraid I will sound like I'm being whiny or complaining.  And maybe I'm trying to trick myself into thinking things aren't as bad.  

But, not today.  Life is super hard right now.  And life is super scary.  And Covid-19 isn't what I'm most afraid of.  Although it is NOT helping.  

We are hoping for a restful night for her and better days ahead.  That's it for tonight.  Tom and I will leave soon to go get some food and take it back to the apartment to eat.  And we are definitely having some wine and beer.  

Thank you - as always - for reading, for caring and loving our girl.  

Much love,
Christy xo

Sunday, May 17, 2020

Back in Boston

Hi all!  I know it has been another long time since my last post.  For the past two months being so "slow" it has actually been quite busy for us.  I've wanted to write so many times, but there's just too much in my head.

Harlie's original hardware removal date was May 12th.  They put the metal in March 6th.  Of course they shut down surgeries since then, so our date has been unknown.  We did know that the 12th was cancelled, but didn't get the new date until last Monday, a week prior to the surgery.  And that was after much discussion with her surgeon (more about that in a minute). 

Honestly, that worked out okay because we didn't think about the original date very well. May 11th was Murphy's 16th birthday.  So, if they still did surgery on the 12th, we would've missed his birthday.  That would've been terrible. And there are limited flights now.  There used to be several flights to/from Boston per day - now there are only a few per week.  So, we would've had to fly to Boston on Mother's Day.  So, all in all, waiting one week worked out better.

I have had some conflicting feelings about this surgery - a mix of excitement and fear.

I am so excited for her that she will be free from this metal.  While it doesn't look that bad from afar, the individual pin sites are still painful for her.  We started with having to clean her pin sites twice a day.  After a while, we were able to go down to one cleaning per day.  Lately, some days we have to clean twice a day again.  It is like her body is trying to get rid of the metal.  So, soft tissue is growing out of some of the pin sites and cleaning it, or touching it causes them to bleed more.

I was terrified of having to bring her out of her protective bubble and put her on a plane and take her to a busy city and into the hospital.  Her surgeon and I discussed this at length during a video conference a few weeks ago.  He said we do not want her to get this virus - and that is so true.  But, this metal cannot stay in forever.  And that is true, too.

We have a known risk with the metal - we are lucky she hasn't had an infection yet.  We have an unknown risk of her getting this virus (or any virus for that matter).  I don't see how we can make the unknown risk more important than the known risk.  After speaking with nurse friends in the field, that has proved deadly for some (people not seeking medical attention out of fear of being exposed to the virus).  And maybe it is better to travel while people are still not traveling as much versus waiting for things to pick back up.  And what would we be waiting for?  And for how long?  Because that metal doesn't care about what is happening in the world.  Her surgeon agreed, so he put her at the top of the list for surgery once they opened back up. 

At first I was thinking we are risking her life going.  We are also risking her life not going.  But, honestly, we cannot live like that.  That isn't living at all.  I cannot live so fearful.  I suppose that is because we have been doing this for 13+ years - not two months like most people.  We have to trudge forward, whether we like it or not.  Normally, I would say "and make the best of it" but that feels like too lofty of a goal right now.

Luckily, Tom was able to get the same Air B&B we stayed in last time, so we knew what to expect.  This sounds like nothing, but when you are already stressed about every little thing, this is a welcome comfort.  And he was able to talk to the owners and they said that the place has not been rented in a few weeks, so that is great, too.

Earlier this week, I received a call from a nurse from Boston Children's Hospital.  She gave me all the details of our pre-op appointments on Monday.  Harlie will be tested for the coronavirus Monday morning at 8am.  She told me they are doing drive through testing and she continued to give me details like, "the patient needs to be sitting behind the driver" and "you will see all the nurses in full PPE, they are changing between patients."  Okay, but, we won't have a car.


Me:  So, I guess we can just walk up and walk through?

Her:  Um, no.  We've actually turned away patients who didn't come in cars.  Some patients take a taxi.

Me: Insert blinking here. Processing information...

If the nurses are wearing full PPE, how is being in a car with the windows down any safer (and for who)?  We are just wearing masks - they have full PPE on.  Does the virus know you are in a car vs. just standing there?  Does something change in the air between a car window that doesn't happen when there is no car?  And you think getting into a TAXI with a STRANGER that OTHER strangers have sat in is safer than me walking my child with heart AND lung disease (who cannot wear a mask, by the way) into the garage?

I get safety and am all for it - trust me.  I am the mom that took my kid out of school in her 2nd grade to keep her safer.  I've been washing my hands diligently (without being told) for YEARS.  I've been avoiding places where kids gather for years.  But, seriously, I don't get this rule.  One size doesn't fit all.  Have a medically fragile child and you'll get that fact.  I'm going to have to come back to that later.

Anyway, she agreed with me that this was pretty crazy, and really made no sense.  She offered to fight it for us.  But, I have to pick my battles.  And, this isn't one I wanted to fight.  I told her the easiest solution was for us to rent a car.  I wasn't super comfortable with taking an Uber from the airport to the Air B&B anyway.  So, this just pushed me over the edge.

My problem is that we don't have gobs of money to throw around.  Times are hard right now and kitchen renovation projects aren't selling like they used to.  And when your one income in the house is very much adversely affected by a tanking economy, we have to be a lot more frugal.  You'd think traveling right now would be cheaper - but our one-way tickets to Boston were the most we've ever had to spend - $263 per ticket (not counting luggage fees).  Plus, parking isn't provided at the Air B&B, so we really didn't want the extra headache of having to worry about a car the whole time.  Especially when the walk is easy and good for us.

Anyway, we will make this work.  I did ask her if there was anything we could do about the 8am arrival time for testing.  Her actual pre-op is scheduled for 1:30 IN the hospital.  And an 8am time is a hard time to have her up and ready and already there.  But she said it takes four hours to get the results of the test and they have to have them back before her 1:30 appointment.  Huh, I didn't know they had four hour tests.  So, I said, got it, 8am it is.

I was thankful for her kindness and reasonableness - I never felt like she didn't understand.  In fact, I feel like she was so empathetic that we had to travel with her.  It was a conversation - not an argument - how refreshing!  I told her I would rather save my battles for more important things.  She gave me her number and told me to call her.  Haha!

Moving on...

Our flight was at 10:30 this morning.

Not a lot of people around.  Such a difference from the way it used to be.  But, of course I'm not telling you anything you don't know.  The airport was depressing.  Getting the rental car was depressing and frustrating.  Tom got a message to go to the fast pick up in the garage.  But then there were signs that said that was closed, so we went back to the counter.  And despite the airport being a ghost town and being the 2nd in line (when we rented a car from here to get the boys from summer camp, we have waited for an hour or more!) it still took forever.  Everyone is miserable.  Whether they are pissed they have to work, pissed they have to travel or just pissed because life is so hard right now - it has created an unfriendly environment.  It really is a sad thing.

In Richmond, only one security area is open, so you go through there and then if your gate is at the other end (as it was in our case) you get escorted by staff through the exit to your gate. 

The plane was definitely not full.

Tom took Harlie duty on the flight.  She is so sweet, but man is she high maintenance on a flight.  So needy with all that oxygen monitoring and stuff. 

On the shuttle bus to the rental car area. 

I promise you, we thought through every extra exposure.  Do we drive or fly?  Driving means a 10-hour trip and having to stop for food, gas and bathroom breaks in busy gas stations.  Do you Uber or take a rental car?  But, taking a rental car means having to take the shuttle.  Ugh.  Thinking through these decisions like a life is at stake is exhausting.

Places that are normally bustling with people...

Harlie taking a selfie with us.

She's happy.  And she's oblivious to what is going on in the world, and we are thankful for that. All this girl is thinking right now is that her life will be better without that metal.  Tom asked her the other day if she was scared or worried about her surgery.  She said, "No, it will be just a little bit of blood."  This sweet girl is tough as nails.

As always, thanks for reading, thanks for caring and thanks for all the unwavering support.  We appreciate it more than you know. 

Much love,
Christy xo

Hardware Removal Day

I'll talk about pre-op day first, then I'll blog about today... Yesterday we did the whole drive through testing thing.  That wa...