Friday, August 18, 2017

Post-Op Day 10

When I got to her room this morning, she was in her wheelchair! She sat up for about 10-15 minutes, which is great!

But she is less communicative than she was yesterday. Barely motions yes, or no. She's not using her hands at all. Its as if her arms are really heavy. Same with her legs.

It has been a busy day already, with occupational and speech therapy coming by. Someone also brought a therapy dog. Harlie didn't seem to care at all, which is very unlike her. She didn't even want to look at the dog. She didn't want to interact at all with OT or ST.  I asked her if she was mad and she shook her head no. But I don't believe her. How could she not be mad?

She is off Morphine completely and they turned off the Dex.

At around 11am, they took her off the vent and put her on trach collar. She coughed up tons of secretions non-stop. Bubbly secretions came out of her mouth, which is a good sign that air is getting past her trach. Her nurse and I literally never left her side, constantly suctioning and wiping secretions from her trach and mouth.

A bit after noon, I guess her body had enough and she started wigging out. She got really restless, wide eyed, purple faced, and sweaty. And she was reaching out for me. We watched her sats go down to 65 in seconds. She looked exactly like she did when she was on bipap last week, like she was frantic. I just can't describe how awful it is to watch her struggle to breathe. If I didn't have PTSD before, I can promise you that I have it now.

They put her back on the vent and she still appeared to be gasping for air. Her mouth opened for every breath. It has now been an hour, and she is still purplish/red. Since she's not on anything for pain (except Tylenol) they were wondering if that was a contributing factor. So, they gave her some Morphine and Versed. And they are going to put her on Oxycodone. She never complains of pain, so we can't count on her to tell us.

It has now been over an hour and a half, and she is finally settled and appears to be comfortable again. They have decided to slow things down a bit. She isn't going anywhere anytime soon, so there's no reason to push her so fast.

Its now almost 5pm and it has been a busy day with lots of docs coming to visit. She's watching tv, which is a nice change. Never thought I'd say that!

They are getting ready to move Harlie to a bigger room, still in the CICU.  When she had that episode earlier today, this room was full. So a bigger room will be nice. Plus, we are right by the main doors to/from the unit and it is very busy and noisy. Doing rounds with people going in and out in the middle of it is not ideal. So, I'm not sad we're moving.

Whew, I am wiped out. Too tired to write anymore. My sister is flying up tonight. So, I'm looking forward to seeing her.

I wish I could thank everyone individually for all you've done. The words of encouragement, kind comments, texts, messages and very generous donations are appreciated more than I could ever say. Being able to focus on Harlie and not worry about how we are going to pay for this is a true gift. Thank you from the bottom of my heart!

Much love,
Christy xoxo

Thursday, August 17, 2017

Post-Op Day 9

So, yesterday she didn't wake up. She opened her eyes a few times, but they didn't focus on anything. At one point we were just talking to her. Then I said, "Harlie, do you want me to leave you alone?" And she nodded her head "yes."  It was pretty funny, because that's SO her. But, nothing else happened for the rest of the day.

They are weaning her off her Morphine. But since she was on a drip for more than 7 days, they are looking for withdrawal symptoms and trying to be gentle. She's been on a ton of meds. So she's being weaned from several.

A nurse came in yesterday to talk about trach training (for me to learn how to do trach stuff). I know she doesn't know us, but trust me when I say I don't need any trach training. She had it for nine years, and didn't have it for two. I could still change a trach in my sleep. But, I'll have to show her and that's fine.

The case manager came by to confirm all of our info to begin the process of insurance changes and ordering the supplies and equipment we'll need. Being trached changes the kind of Medicaid program she needs. And thinking about what we'll need to go home, and what will need to be at home, for us to take her home makes us so sad. We got rid of everything. It had been two years, and we never thought she would be trached again. It is very hard to start to think those things through, so I stop. We aren't even talking about going home yet anyway.

Tom and I got up early this morning so we could be at her bedside at 7:30am. You can't come or go between 6:30-7:30, both morning and night. That is "distraction free time" for the nurses to give report.

Anyway, when we got here, she was much more awake than yesterday. The night nurse washed her hair and braided it. I went to take a picture, and surprisingly Harlie smiled! I couldn't believe It! She is so amazing.

She's trying to communicate, but she can't talk because of the vent and her general weakness. And she can't sign yet because her hands and fingers are still really weak. She hasn't gotten control of them yet. She mouths words, but I just can't understand what she's trying to tell me. I can tell she wants water. But they said she can't drink anything yet. She has too much muscle weakness to let her swallow. So we are putting some water on a toothette and putting that in her mouth.

Tom told her she had a trach again, but we don't know if she understands. She's pretty groggy still. Tom had to leave a little after 8am. And that was hard. If we had known, he would have stayed and worked over the weekend, coming up later so he could be here during the next couple of days. But we didn't know. And when your child gets a trach in the middle of the night because she couldn't have lived without it, you can't help but want to be there. So, we made the best decisions we could at the time.


She is starting to cough a lot. And she has a ton of secretions. But she is completely silent while on the vent. And I just want to suction her, but they have a whole process, so I have to be patient. I totally remember feeling this way in the past, too. It's just so hard when I want to help her, but I can't.


They just rounded. They are going to start weaning her vent more. The goal is to get her on trach collar tomorrow. I know she'll be happier then. But they want to get her on less to no Morphine first.

I asked them how much longer they think she will need to stay in the hospital (told them I will not hold them to it as I know things change) and she said 10 to 14 days would be her guess. ☹

We did trach care and changed her trach ties this afternoon. Still remember how to do it...

She's had a low grade fever today. Hopefully that won't last long.

Dr. Haas came by and worked on ways to stretch her jaw. He said that she is making him think outside the box. So typical Harlie. I told him she doesn't discriminate, she treats all her docs the same, and never plays by the rules.

So, he is trying these tongue depressors. Starting with three and will work up to five, I think. She's tolerating it right now, so I know she's still loopy. Pretty sure she's going to throw those out when she is more herself.

Some friends visited today! Our friends Dale and Patty and their son are visiting some family here and stopped in to say hello. It was so nice to see them!! They made me laugh and that's always good! Thanks Dale and Patty!!

That's pretty much it. Overall, slow progress, which is better than no progress at all. I'll certainly take it.

I'm sure tomorrow will bring more excitement as she becomes more herself.

Thank you, as always, for continuing to keep Harlie, and the rest of us, in your hearts, thoughts and prayers. We are staying strong and trudging forward, one baby step at a time. And we couldnt do it without you!

Much love,
Christy xoxo

Wednesday, August 16, 2017

Post-op Day 8 and Trach Change Day, AM Update

It is so weird to have such mixed feelings about today. I hate seeing her in this paralytic state, so I'm SO looking forward to her waking up. But, I'm really not looking forward to her being in pain from the various sources (pressure sore, IV sticks, bloating, mouth, ear, jaw, etc.). And, of course, the realization that she's trached again. How weird must that be... to wake up almost a week later and wonder WTH happened?

As much as I've worried about it, she will probably accept it and move on, as she always does, because she's so amazing. And that's pretty much how she's handled everything so far.

It is 10am and they finished rounding. We are just waiting on ENT to come and do that trach change. I've been told they are going to put the same kind and size in that she has now, a Bivona 4.0, cuffed. The cuff can be inflated or deflated. While inflated, it fills all the space around the trach tube and her trachea, so that the only air that goes in and out, is through the trach. And it is inflated while she's on the vent becuse it is easier to manage her vent settings when no air escapes.

However, when off the vent, the cuff can be deflated so air can escape, and go through her vocal chords, allowing her to talk. This is our hope.

Our nurse, who is great and was her nurse yesterday, said she can have education come by to talk to us about the vent, should she not be able to come off. Clearly, it is our hope that she can breathe on her own and not require the vent. For one, life will be better for her. And two, her cardiac physiology does much better without ventilated breathing. So, I told her we aren't ready to talk about that yet.


ENT just arrived and are setting up for the trach change. Its pretty crazy how big of a deal this feels when we've done hundreds of trach changes before.


Its done and all went well. They turned off her paralytic and now we just wait for her to wake up.

I'll update again later.


Tuesday, August 15, 2017

Post-Op Days 6 and 7

August 14 , Monday

Thankfully, she seems to have settled down and we are starting to see small amounts of improvement.

They took cultures from her trach secretions, blood and urine, and all grew something, meaning she has infections. That explains her really bad night Saturday night.

Infectious disease just came in and they are going to figure out her antibiotics again. They said she has staph aureus in her blood.

Since they put her on Vanc and Zosyn, she has not had a fever. But her WBC were still a little high. So, they are going to take blood cultures every day until they show negative for 48 hours.

Dr. Padwa and Dr. Haas came by. Dr. Haas put some braces on her loose bottom teeth to help them heal and hopefully prevent her from losing them. She will go home with them and our local orthodontist will remove them when its time.

At this point I feel like I have a really good relationship with her docs. They seem to be ok with my sense of humor. So when I saw him I smacked him on his arm and said, "She lost a permanent tooth and I'm mad at you!" Because he was the one that said it would be fine. Of course I had to tell him that I know they don't know all the answers and that Harlie doesn't follow any rules. And that I wasn't really mad at him. Although, I'm pretty sure he knew that.

Dr. Padwa and I joke around that she regrets ever taking Harlie on as a patient and that she will never operate on her again. She told me that Saturday night/Sunday am her and Dr. Resnick were texting about them not being able sleep because they were so worried about Harlie. Harlie is surrounded by such a great group of doctors. Every single one has been so great, caring and compassionate. And Dr. Padwa says she's not going anywhere and that she's committed to Harlie. Which, I already knew.

While headed outside for lunch, I ran into Dr. Mancuso (who was with her on Thursday night when she got the trach). He is wonderful. And he told me that Harlie got the right mom. Which, is always nice to hear. And this morning (Tuesday) he stopped by her room to say hello and see how she was doing. Love him.

We also spoke to the hotel about our stay. They were so nice. We had to move rooms to get a cheaper rate ($210 per night). And they put us just a few doors down so it would be easier to move. And they threw in free breakfast whenever we want it. Which, has been/will be each day. ☺

That night we went to the Red Sox game at Fenway. We have never been. And we didn't have tickets. But, a friend of a friend of a friend... let us in. Shhhh. So, we just walked around and watched the game from different areas. The Red Sox did not have their best game, so there were plenty of empty seats. Honestly, we just enjoyed being there, regardless of who won. It really is beautiful. And we had a great time.

August 15, Tuesday

We were going to wake up early and see if we could pay to do a yoga class down the street. But, when the alarm went off, I just couldn't. The kind of tired you get in a hospital is different than a normal tired. So, we went back to sleep.

After a couple of hours we got up, had breakfast and went to the hospital. We caught rounds and then checked out the gym across the street. When Maggie was here she said she overheard someone talking about how they offer free membership to parents who have children here. So, we went to check that out.

It was good. They didn't have bumpers to do deadlifts, so that was weird. But we managed. And we did a full workout and we both felt like ourselves again. So, that's really good.

As far as how she is today, she's the same, and no worse, which is good. They had to stop her feeds again because her belly was getting really big and her body wasn't digesting it. Since she's on that paralytic, it really slows everything down. They are constantly adjusting her Lasix (direutic) to try and get a good fluid balance. Her belly looks terrible from the fat graft.

Forgot to mention that Dr. Padwa said they were not going to stretch her jaw with that device anymore since that was so hard on her teeth. And they are well aware of my feelings about her teeth. So, they put a soft wedge thing in her mouth today. Its attached to the string in her mouth.

They also pulled her PICC line as it was a potential source of the blood infection.

So, tomorrow is the big day! They will change her trach (hopefully in the morning) and then turn off the paralytics, to let her wake up. We don't know how long it will take for her to wake up. It's different for every person. So, Tom and I will be glued to her side all day. I will be so glad to see her open her eyes again. I feel like I haven't "seen" her in so long. It is so unsettling to see her just lying there like that. Plus, they are having to keep ointment in her eyes because she doesn't close them all the way.

And the bottom line is that we can't see where she is in her recovery until she wakes up. The sooner she can breathe on her own, the better.

Tom is going back home on Thursday. And my sister, Sandy, is flying up Friday afternoon. I think I forgot to mention that the visitor rules are different in the CICU than they are in the Medical/Surgical ICU. In the CICU there are only two people allowed bedside. In the MSICU, they were fine with four of us. Luckily, she didn't get moved to the CICU until after Maggie left.

Okay, that's it for now. Hopefully all will go well tomorrow. While I'm excited to make progress, I'm not looking forward to her finding out she has a trach again. I don't know how she could possibly understand. I feel so bad for her. She loves the pool and getting things off the bottom and she was showering independently (both of which will now be out of the question). While I am more thankful than words could say that she's HERE, I am so, so sad for what she's lost (and for what we've lost), again. One day at a time, I suppose.

I just want to end by trying to thank you all again for all the AMAZING support you've given. You have all been so generous, in monetary support and love. I just don't think I'll ever be able to express how grateful we are. We are feeling the love and it is keeping us strong!! Thank you so much!!

Much love,
Christy xoxo

Sunday, August 13, 2017

Post-op Day 5

It's so hard to believe we are only in post op day 5. Wow. It's been a busy 5 days.

I had such a hard time sleeping last night. My stomach hurt so bad. And so many things were going through my mind. So hard to shut those thoughts down. I finally fell asleep and woke up early. I called her nurse to see how she was. I'm so happy we got the same nurse we had yesterday. It really makes such a huge positive difference to have nurses who repeat.

Anyway, she said Harlie had a really rough night last night.  But that she had settled down a bit.

Her really low blood pressures could indicate a big infection. So they changed her antibiotics again. Now she's on Vancomycin and Zosyn.

They are giving her some blood. They could not move her at all last night, because she was so precarious. So, she has developed a pressure sore.

And she lost a permanent tooth this morning. Ugh. Do you know how long a healthy, permanent tooth is? It looks way to long to fall out, trust me. And more are loose. Really, I just can't.

They are moving her to the cardiac ICU today. They are having to consult with them so often now that it just makes sense to be there.  She's less post op care and more cardiac care now. That should happen this afternoon.

When we went to Walgreens to print some photos out for Harlie's room, I picked up some cards and we played in her room. We spent more time in her room today, but we wanted to.

It's now 5pm, and she's now in the cardiac ICU. Maggie just left to go back to Richmond. I'm going to miss her. She was a really good distraction for me.

Maggie and Noah saying goodbye.

Harlie has a temperature probe that goes in her nose and sits in her esophagus. They could not get an accurate reading from anywhere. So, they are going to leave this in until she goes some time with no fevers.

I just saw her pressure sore and it made me flinch. Her nurse said that it doesn't blanche (?) which means it is deep. Ugh.

They are stopping the jaw stretching until we discuss things tomorrow. It just seems to really upset her balance overall. She's had difficult spells after each one. She just needs some rest. And those poor freaking teeth need to heal a bit.

Earlier today I asked her nurse to tell me something good. I'm usually pretty freaking good at finding a silver lining on my own. But this whole hospitalization has thrown us for a loop. We are in brand new territory all around.

Anyway, she said that she had twice the amount of secretions yesterday than she has today. And that she is on less oxygen. She was on a lot more yesterday. And I think her vent settings are a bit softer today vs. yesterday. So, overall, she felt that her respiratory status was improving a bit. So, that's good. We'll take it.

We have not told the boys about what's going on yet. They are having fun with Grandma, friends and family and we don't want to worry them yet. It will be easier to tell them if we can say she's better. And we aren't there yet.

Tom is going to stay here through Wednesday, at least. We both want to be by her side when she's able to wake up.

Forgot to mention that we went out on a bike ride this morning. They have these bike share things where you can get a bike from any station and return it to any station. We went through the park. It is amazing how I can feel almost normal for a second. Tom is really good at distracting me and making me laugh, even when I just want to cry. Plus, the sunshine, fresh air and exercise is so rejuvenating.

Lastly, we are truly overwhelmed with all the love and support we are getting. I just can't get over how many people care about her and us. Lynda posted on Facebook that We Heart Harlie & Friends has already raised a lot of money to help us pay for some of our expenses. Our community has already been so generous. The words "thank you" always seem so inadequate.

As much as we love Boston Children's Hospital, it is shocking that they don't have a better set up for families who are from out of town. The Ronald McDonald house is for cancer patients only. And the two houses they have here have been completely booked every time. I completed an application, and they put us on the waiting list. So, we'll see.

Anyway, for now we got the medical rate at our hotel and they threw in free breakfast, so that's cool.

Okay, it's getting late and we need to eat. Thank you, truly. We could not survive this life without you.

Much love,
Christy xo

Post-op Day 4

Yesterday, (post-op day 3) was rough. I had not slept at all the previous night and being tired on top of being sad is not a good combination. And I just couldn't write.

Although I had been really good and was keeping myself together fine, until the anesthesiologist who intubated Harlie for the surgery came to visit. She just has that personality and really sweet face that makes you comfortable. Plus, Harlie's nurse was so sweet and compassionate, too. As the three of us were talking, I asked her how long it took to intubate her under perfect conditions (she was well and breathing on her own). She said minutes, and that she got lucky.  Somehow the realization that she is going to remain trached for the foreseeable future hit me, and I started to cry. Once I started, I had a very hard time stopping. Then Harlie's pediatrician called her nurse to check on her. Seriously, how sweet is that? Her nurse gave me the phone so I could talk to her. Once I heard Dr. Sutherland's voice, which is also very sweet, I started to cry again. I couldn't talk to my mom, my sister, or any of my friends because I knew I would cry. And I had to keep it together as much as I could.

There is so much going on here, medically, and it is hard to be able to comfort myself thinking/hoping that this trach is temporary. The fact is that no one knows. It's way too early to tell. Let me try to explain...

The ENT who trached her and Dr. Padwa said that she should never be intubated again. They felt that she would need to be trached preemptively for every surgery from here on out.

I think I might have mentioned in a previous post that Dr. Resnick had to remove one centimeter of bone from her jaw.  My first thought was is that going to affect her airway. We worked so hard to make that jaw longer, and now it will be one centimeter shorter. I remember talking about adding millimeters. And one centimeter means taking away ten millimeters! That's a lot!!  Anyway, he said that it was up near her ear, so he didn't feel it would affect her airway.

Also, she's had a lot of lung issues and pneumonia. And I've never seen her go downhill so fast. Plus, I noticed that when they look at her chest xrays, no one has a major reaction. What I'm trying to say is that what we know about her lungs didn't match how bad she was doing (in my opinion). So, I can't help but be fearful that there is/are additional factors contributing to her decompensation.

So I asked Dr. Padwa about the centimeter Dr. Resnick removed. She apparently went home and thought about it. Because when she came to visit last night, she said she always listens to moms, because they are usually right. And then she said that maybe it did affect her airway negatively. Maybe her clearance was smaller than we knew. And this just tipped her over.

So, to summarize, this is what we know:

1. She has a very small airway as it is. She also has congenital cervical spine fusion. This makes it impossible for them to put her head back enough to get a better angle when trying to intubate.

2. Her airway itself is irritated, swollen and bloody from the intubation tube, jaw surgery and tooth extraction.

3. Her lungs have atelectasis (collapsed) and most likely she aspirated blood.

4. The centimeter removed may or may not have affected her airway structure (meaning the jaw bone, not the trachea itself) causing an upper airway obstruction (like she had for most of her life), causing her to struggle to breathe.

So, we have to wait until we can start to eliminate some of these issues to see where she settles. The problem is that if I think about #4, I know what that means. It means more jaw surgery before decannulation. And it makes me think about how she's going to talk. We don't know yet what kind of airway she has. Air has to be able to get past the trach, go through her vocal cords and out her mouth for her to be able to talk. An upper airway obstruction makes that much more difficult, if not impossible. We have to be patient till we find out the answers to so many very important questions. And that's so damn hard.

As to how she's doing now, she is under general anesthesia so she can't move on her own.

They gave her a PICC line yesterday. She only had one IV, and since she MUST get sedation meds, it was too risky to not have another access point (what if they lost her IV and they couldn't give her meds?).

Her lungs are still hurting, but what they would normally do to help the progress and get rid of secretions (chest pt, her coughing) they can't do because of her freshly placed trach. So, she just has to stay on course with antibiotics, oxygen and ventilation until her first trach change.

Her first trach change is Wednesday, I believe. After she gets that change, they will let her wake up.

She spiked a fever today, so they added an additional antibiotic.

They have not been able to restart her feeds. Her body just isn't ready for that. It's just so crazy because she has had virtually nothing since Monday night.

Since she is under, plastics is stretching her jaw for us. They are using a clamp that they use in the operating room. They put it in and stretch her jaw for 15 minutes, twice a day. They are giving her a bolus pain med prior to, because her blood pressure indicated that she was in pain when they did it the first time.

During the first time, they noticed that she has a loose tooth that bleeds when it is touched on the bottom of her mouth. Its a permanent tooth and I'm guessing its unstable because they removed the one next to it. Regardless, I almost lost my stuff when I found out. It is one of the bottom four teeth that the surgeon said didn't have a lot of bone under them. I just can't. It was about to be the straw that broke my back. But when plastics came by I asked him about it and he said it would heal and likely be fine. They are trying not to put the clamp on that tooth. Ugh. This whole thing is killing me.

I don't think I mentioned that Tom flew in late last night. We spent the morning at the hospital and left early afternoon. Maggie's boyfriend (Noah) met us out and the four of us hung out and walked around. Then we got dinner.

After Tom and I got back to the hotel, I called to check on Harlie. The nurse said Harlie had a busy evening and she was about to call me. What?!

Her blood pressures are just too low. They started dopamine, but apparently that's not working for her, so they are going to start epi or something. She can't be moved because her blood pressures drop. She spiked another fever. Her stomach is not doing well, and a lot of blood and stuff are still venting out of her gtube, so giving her Tylenol isn't working. So they are trying a cooling blanket.

She said a bunch of other medical stuff, but I can't remember it all enough to explain it.

The fact is that she is really struggling and my stomach hurts thinking about it. Having a medically fragile child gives you an introduction to a whole new world that most never even know exists. I have met so many moms who have children with rare, complicated medical conditions. I've watched so many of them suffer through surgeries, hospitalizations and many sicknesses. And I've watched many lose their children. Its awful. Its heartbreaking. But it happens.

So I KNOW there are NO guarantees. I know that every time we take her into surgery. I HOPE more than anything that she will bounce back. But I don't KNOW. So, I'm scared. She's very sick and she has a lot going on. None of this was expected. And I'm having a hard time with it all. And I miss HER.

Look at her just a few weeks ago.

And this is her now.

Hopefully they can get on top of that fever and she will settle tonight. I'm sorry this can't be more positive. I'm looking for it, I promise.  We just aren't there right now.

Thank you so much for the love and support. I know this isn't easy for you, either.


Friday, August 11, 2017

Post-op Day 2, Part 2

Unfortunately, things took a turn for the worse again.

Harlie continued to struggle all day. It was agony to watch. Her episodes were panicked. She would look at me with these wild eyes like she was trying to tell me something (probably help me). And she would reach out her hand - ugh. It was truly awful. So, so hard to see her like that. Communication was in gestures only. She was sweaty and exhausted and working SO hard to breathe. All I could do was tell them I was worried. This was officially the worst she's ever been. On post-op day 2, we left "been here before" and entered "I've never seen this before and its scaring me." They ended up giving her some sedation meds to try and calm her down and Fentynl for pain. 

Once she seemed calm (6:45pm), we left for dinner. At 8pm I called to check on her. Her night nurse said she was calm at the moment and she would call me if needed. So, Maggie and I headed back to the hotel. 

At 9:40pm, I got a call from the hospital. The ICU doc called to say Harlie had taken a turn for the worse and that he was concerned and needed me to come in. In all her years (11 next month) I have never gotten called back to the hospital. 

The second I hung up the phone, Maggie requested an uber and we ran out the door. 

We got there like 8 minutes later, and Harlie looked truly awful. It had become somewhat emergent that they re-intubate her to be able to ventilate her better and give her body some rest. And that they were taking her to the OR soon. I signed all the consent forms and struggled to be patient. 

Dr. Padwa came in, as did another one of the plastic surgeons on her team. Dr. Padwa was getting in bed when she got the call, and she rushed in. Love her!  And I love that they both came in and care as much as they do.

Dr. Padwa
It felt like forever till they finally took her down. While waiting I jokingly asked the surgeons how they ever have a life if they come in for their patients like this. Dr. Padwa said this doesn't normally happen to their patients. I said, "you mean not everyone is like Harlie?! I joke when I get stressed. And I was so super stressed. They just couldn't take her fast enough. 

At close to 11pm, we headed down to the OR.

We got on the elevator and the doors closed, and then opened back up. The doc hit the doors close button and they closed, and opened right back up. This happened like 4 times! We had to get out of that elevator and get on a different one!

The team was so annoyed. I jokingly said, "what was that elevator number?" And a woman said, c290, or something like that and was already on the phone with maintenance telling them they had a critical patient in a broken elevator. 

We walked her as far as we could go, then went to the waiting room. The doc said it would probably take 15-20 minutes. 

Soon after that an ENT doc came in to introduce himself and tell me that he was there as back up, just in case.

Minutes creeped by, 30, 35, 40... I started to get very worried. I'll be honest, I was very scared. She looked so bad. What was going on in there???

The lonely waiting room in the middle of the night. 
Just a few minutes shy of midnight, the ICU doc came out. I tried to read his face. He said she was fine (whew!!!) But, that they had to give her a tracheosotmy. 

Do you know what happened on August 11, 2015? She was decannulated (meaning the trach came out). And two years later, minutes shy of her 2nd Decanniversary, she was re-trached. 

I just can't believe it. There are 365 days a year. And she got decannulated and re-trached on the same date (minutes shy, I should say).

He said he had considered attempting to re-intubate her bedside. He was so glad he didn't because she wouldn't have made it. 

Removing the bipap mask made her unable to breathe, meaning that the doctor only had 10-15 seconds to try to intubate her. She said there was too much swelling, blood and irritation to do it in that time frame. They would stop, put the mask back on and let her recover. With each attempt, she deteriorated faster and took longer and longer to recover. In addition, each "recovered" point became less and less ideal. 

They tried both her nose and her mouth, two times each, I think. Then it became too risky. They were gravely concerned that her heart and lungs just wouldn't be able to handle any more stress.  The team worked together to make sure they had exhausted all options. No one wanted it to come to this. But they had no choice.  

Considering how feisty she is, they have to keep her sedated until they can do a trach change, in five days. If she were to pull that trach out, it would be life threatening. So, they stitched it in and are keeping her under.  While I hope that this is temporary, of course, they said we would most likely go home with the trach. But I can't think about that right now. 

Her lungs are so done and need some time to recover. Can't remember if I already said that they believe she aspirated blood and her lungs are angry. 

It was very hard to call Tom and tell him. And I am so afraid of what Harlie is going to do when she wakes up, and realizes she has a trach again. All I can think of is how she was trying so hard to do a flip under water in the pool. It breaks my heart to think of it. 

I am trying so hard not to be sad (which is both ridiculous and impossible). But, fearing her loss as those minutes ticked by was agony. I'm so sad that she's so sick and that her body is so incredibly fragile. But I am more thankful than I could ever express that she's safe in the hospital right now. 

This team is amazing and I am so grateful to each of them for working so hard for her. They moved her from a private room to an open bay so there is more room for stuff and so she can be more closely monitored.

Once she was settled and I had answered all questions, Maggie and I left the hospital (2am). I couldn't sleep. It is just so hard to believe that she was at her all-time best on Monday, and at her all-time worst by Thursday.  We are truly devastated.

But, we will figure this out. Day by day, hour by hour, if need be.  I have hope that she is finally getting the rest her body needs and that soon she will start to get better. 

Tom booked the first flight back to Boston, which isn't until tonight. It will be good to have him here for the weekend. Clearly, I have no idea how long Harlie will be here.

So, it sucks for sure. But she's here and that's what matters most. Please don't worry about us. We will get through this. We are not out of love nor fight, especially Harlie. She's already got a reputation around here for being such a fighter.

Thank you, from the bottom of our hearts, for your love and support.


Post-op Day 2

My, how fast things change. Left her last night looking great. And when I called the night nurse this morning to see how she did over night, it was a whole different situation. She said she had a rocky night and was up all night coughing and had to go back on oxygen. She said she had finally settled a bit and fell asleep at 5:30am. Oh, my poor girl. So hard to think of her being awake and uncomfortable all night long.

When Maggie and I got to her room, she was a mess. She was very upset because she had to go potty and didn't want to go in the bed. Unfortunately her nurse didn't quickly understand what she was signing. It got Harlie so messed up from a medical perspective (meaning it got her out of breath and she just couldn't recover) that her sats went down to 53! She looked terrible and her chest and shoulders were really moving with every breath. She was working so hard, and she just couldn't recover on her own.

So they had to put her on bipap via a face mask, which she's never had. You could tell she was EXHAUSTED because she didn't fight it going on at all.

They took more xrays and they said she has something brewing (pneumonia) in her right lung. It could be fluid related or maybe some blood got in there.  They gave her lots of fluid during her surgery and she is way up in weight. She came in at 54 pounds and is now 59, and hasn't has any food really since Monday night. They started her feeds slowly yesterday, but they stopped them when she had that episode and I don't know when they will restart them. So, she's very fluid over loaded. She's on Lasix, which is a direutic to help her pee and get rid of some fluid.

So, she's staying in the ICU tonight for sure. We'll talk about tomorrow, tomorrow.

So, it's 4pm and she just had another episode. She took off her mask and when I tried to put it back on she smacked it out of my hand. Within seconds she was working so hard to breathe. She looked horrible. She looks like she has a lot of things that are making her hurt/uncomfortable and she just doesn't know what to do. I think she hit the respiratory therapist. She is a very unhappy girl today.

This was all so much easier when she had her trach. Can't believe I'm saying that. But I can't help but be nervous about what happens if she doesn't tolerate the bipap mask. She just looks so uncomfortable.


Honestly, the video doesn't do it justice. Her chest and shoulders were way worse when she took off the mask. Within two minutes of going back on bipap, she was sleeping. Her body is so very tired.

Ok, the day isn't over, but I'm wrapping this up. Will fill you in on the rest tomorrow.

Thank you for the love. Can't possibly tell you how much it means to us.

Thursday, August 10, 2017

Post-op Day 1

Hi! I was way too tired yesterday to write, so here is a recap of yesterday.

In the morning, when we came around the corner and headed to her room, a doc stopped us and asked if we were Harlie's mom and dad. He was an ENT doctor. He said the plan was to extubate her that morning (they had been able to wean her breathing support settings down to bare minimum) and that he was asked to be on standby, just in case. So, he had some questions about her airway and her cervical spine issues. It was so weird because I haven't been asked about her cervical spine in years, nor do I remember which vertebra is fused.  Anyway, he said that if they extubate, and she can't handle it, and they can't get it back in, that he is prepared to trach her bedside. While that was a bit unsettling to hear, I didn't think it would come to that. Can you imagine? Awful to think about.

Anyway, all went well. It was a little crazy waiting for anesthesia. They wanted her to be asleep until they showed up, then awake immediately, so tiny amounts of drugs were given often. She woke up when they were told anesthesia was 2 minutes away. So, we got to say hi and tell her the tube was coming out. Tom told her to be brave and she shook her head yes. 

While it was awful to watch, all went great and there were no emergencies!

Extubated and trying to smile!
This whole process was a brand new experience for us. If she still had her trach she would receive all the breathing support she needed with no drugs and could be awake and happy. Which is how it normally was for every single surgery she's had so far. 

Also, keep in mind that her last surgery here resulted in big pneumonia and she was on the ventilator for like 6 days. I just didn't want that to happen again, especially while intubated!

Anyway, her voice is very soft and weak. So you have to be close to her to hear. And usually we have to ask her to repeat herself, so sorry Harlie! She asked Tom, "Daddy, when can I sing again?" What I wanted to say is, "You really couldn't sing before..." haha! But boy, does she like to try. 

Maggie flew in that morning. Coincidentally, she is dating a guy who lives in Boston.  So he picked her up and we all met at one of our favorite restaurants (the Squealing Pig) for lunch. 

That afternoon, Dr. Padwa came in and showed us how to use the therabite. That was agony. But she said we have to use it as much as possible or we will end up in the same position.

Somehow I need to come up with a way to show Harlie what it's doing. If she understands I think that might help her be a willing participant. 

I ended up telling her we will do it once every hour. And by 8pm, she was tolerating 2 squeezes for 10-12 seconds per squeeze. A huge improvement in just a few hours. She is such a trooper.

Well, Tom left tonight to go back home. So, that's sad. Luckily Maggie is here so that's good. We all went to an early dinner together at a pizza place called Otto. 

While it was so good (eggplant, ricotta cheese and fresh basil) I had a hard time eating. I just feel like I'm super nervous (even though I'm not). Honestly, I think it's PTSD. My nervousness and anxiety comes in waves and not necessarily in times that make sense. It's weird. 

Then Tom ubered to the airport and Maggie and I realized the walk back to the hospital felt like too much. We were exhausted. The hotel is just under a mile to the hospital. And we walked there in the morning, then walked to lunch (about half a mile), then back to the hospital, then back to the hotel to get Tom's stuff and drop off Maggie's stuff, then to the restaurant. Whew. Normally, this wouldn't wear me out. But today it did. So we ubered back to the hospital. 

Her night nurse had to pull out one of her IVs. Harlie told her to come back at midnight, when she was asleep. Haha! I told her she would wake up and that would be worse! So the nurse took it out. She left the room and when she returned a few minutes later, Harlie looked at her angrily and said, "you should've done it at midnight when I was asleep." Haha! She is something. 

So, we did the theabite 2 more times and gave kisses goodnight. Today was a good day. She was extubated, then she was able to come off oxygen. They said she was staying in the ICU tonight and We'll talk about tomorrow, tomorrow.
Here is the last picture I took. For some reason, I love it. I think she is amazing. 

Thank for all the love and support!
- Christy xo

Tuesday, August 8, 2017

Surgery day

Hi! All is well here so far. She had a good night's sleep and woke up early and in a good mood. Yesterday we had to wake her up at 4am, and today at 4:45am, and no complaints from her.

All went well during check in. Risks were nerve damage to her forehead and right eye and airway issues. They said they had a trach ready, should she need it. They plan on keeping her intubated because she has an increased risk of excessive bleeding due to her cardiac issues (her circulation is different because it's been "rerouted"). They are going to wait and see how she does. So, no hard plan as to when they will extubate (hopefully this afternoon) and they might extubate in the OR to provide the safest conditions. Time will tell. But I'm betting she'll be fine and won't require that.

She went back like a champ, in a good mood with ZERO stress, worry or anxiety of any kind.

11am: Dr. Padwa just came out to say she was done and Dr. Resnick was finishing up. They took a fat graft from her abdomen to put in that area. She said she thought that was the hardest part - finding fat on her! I am NOT surprised. Tom said he'd be happy to donate some of his, but they didn't take him up on his offer.

12pm: Dr. Resnick just came out to tell us he's done and that all went well. He thinks her nerves are going to be fine. She might have some muscle weakness in her eyelid and right upper cheek, but he expects that to go back to her normal at some point.

They removed about a centimeter of her jaw bone to release it from her skull. That's where they put the fat graft to try and create soft tissue scarring to try and help prevent it from fusing again. Although both surgeons said it could grow back and we would have to do this again.

He extracted 8 baby teeth and the 2 permanent canines that were sideways in her chin. The permanent ones were too far gone to try to save (as you can see in the pics above and  below). One, it would've taken heroic efforts (which no one wants to ask that of her because it would be very painful) and two, in the attempt to save those two, it could cause the loss of other teeth. And really, there just wasn't room for them.

The unfortunate result is that the canine that the arrow is pointing to was helping to support the tooth beside it. So, now that the support is gone... We'll just have to see.

The other unfortunate situation is that she will get a Therabite to help her open her mouth and try to keep her bone from refusing. This is an image I found. I have no idea if this is the same device she will get.

And her front teeth on the bottom have only a little bit of bone supporting them.  Also, she has no molars on the bottom in the back (circled in the xray pic above) so there's not a lot of support overall to distribute the pressure when she bites. And it might be really hard on those front bottom teeth. Add that to the mix of losing the support to that bottom tooth and I don't know how I'm going to make her use this thing! It's damned if you do and damned if you don't.

Here are her teeth...

Wow, those permanent ones look scary. That's gotta hurt! She has now lost at least 17 teeth in the operating room.

5:30pm: So, no extubatation today. She's on too much breathing support (ventilator). They think she might have some atelectasis (collapsed lung) and/or some blood got into her lungs.

The major problem with that is that she really shouldn't wake up. Generally, you don't want to be awake with a tube up your nose and down your throat.

Soon after she got moved into the ICU and we got to see her, they took a chest xray and said her tube was way too high. So they had to push it back down. There was still a lot of people in her room and Tom and I didn't really want to get in the way, so we left to get some lunch.

She seems to go from resting to awake very fast and it's very unsettling to watch her struggle. She is trying to sign water (her arms are restrained to keep her from yanking out her tube). She spit out the gauze in her mouth. So we wet a toothette and just put a little in her mouth.

Her blood pressure is low, they have been working on that all day.

She just moved over and woke up. The nurses came running. She really can't (meaning she shouldn't) pull that tube out because its just too risky of an airway (hence the critical airway sign above). Several people came to help and  Tom calmed her a bit, but they gave her more meds. Its really crazy how much they've given her already (Dexmedetomidine, Propofol, and another one I didn't recognize).  She's on a Fentanyl and Dex drip now.

She totally understood what Tom said and she tried to talk, but no sound came out (the tube goes through her vocal chords). She is way more aware than I would like her to be. The respiratory therapist just came in and said she had to increase her breathing support. NOT the direction we want to go. I really hope that they can safely extubate her tomorrow. She would be so much more comfortable. But I guess at this point her lungs have to be able to support her. Ugh. Her poor lungs. As strong as they've gotten, they are still so fragile.

Okay, so things aren't great. This is never easy. It hurts so bad to see your child like this. Hopefully tomorrow will be much better. I'm already hating that Tom has to leave tomorrow. I focus on Harlie, and he focuses on me.

So, I'll leave you with this pic from last night. Tom was removing her toenail polish. I love her little giggling face.

As always, thank you for the continued love and support!


Monday, August 7, 2017

Gearing up for Jaw Surgery #5

Hi all,

So, we are off to Boston again. The past few days have been a little crazy.

On Thursday morning I got a call from the scheduler (Amanda) for Dr. Padwa, one of Harlie's  surgeons. She said that the cardiologist at Boston Children's did not clear her for surgery on Tuesday.  He said he wanted some recent studies first, one of which was an echocardiogram. She was trying to get her in during her pre-op day at the hospital on Monday, but their schedule was full that day. I tried hard not to panic. But this has been scheduled since January. There are two surgeons involved with this surgery so getting them both on the same day meant having to shedule far out. I couldn't bear to think of how much longer we'd have to wait if we had to reschedule.

Late that afternoon I spoke with Amanda again, and she told me she had no luck. We entertained flying up on Friday, as there was room in the schedule that day. But, all the flights were booked. My older brother, Bruce is a pilot, so I called to ask him if he could fly Harlie and I. Unfortunately the plane he has access to is down for maintenance. So, Friday wasn't an option. So, I asked if I could get her in for an echo here locally, and I could bring the images and report with me on a disc. She said that she had already suggested that but something about echoes not always turning out, so she didn't think that would work.  I went on ahead and emailed her local cardiologist anyway, just in case. He said he could get her in.

That night was a difficult one for me. Life has been so good for the past two years that remembering how complicated her heart is felt like a punch in the gut. It's stressful leading up to a surgery when everything goes smoothly. This obstacle made it SO much worse. 

The next morning I woke up and sat in front of the computer and I emailed the cardiologist in Boston. I love how accessible all of her doctors are and how quickly they respond. I updated him on what's changed since he last saw her four years ago (decannulated two years ago!). And I also shared how I felt about this surgery.

I don't think I've told you what surgery she's having. Five years ago they removed her fibula from her right leg and used it to create her jaw bone on her right side. Four years ago they distracted that fibula graft (put pins and rods in her jaw on both sides, and we broke the bone each day by turning screws so new bone would grow in between the breaks). That fibula has continued to grow and fused to the base of her skull, preventing her from being able to open her mouth. I was thinking that growth destroyed her TMJ, but now I think I remember she never had it on that side to begin with.

Anyway, we don't know how long it's been that way. I'm guessing a year. Funny how it wasn't as noticeable as you might think. Her mouth has always been very small. Just the sheer thought of knowing that her mouth is fused shut freaks me out. Access to her teeth is impossible, and any kind of emergency that involves her breathing scares me to death (car accident). She also has two rows of upper teeth since her permanent teeth couldn't come in over her baby teeth. There's just not enough room in her mouth. So they are going to extract a bunch of teeth.

Soon after I sent that email to him, Amanda called to tell me that he wanted her last cardiac cath report. Her last Cath was 3.5 years ago in DC. I called there and got someone to listen to what I needed. It isn't so easy to get those kinds of reports (with images). They are huge files and can't just be emailed. It took many phone calls (including Amanda calling the person in DC and talking her through uploading it to Boston) and lots of time and hard work (plus many thank yous!) and they got it done! A hurdle crossed!

I also called and got her in to get an echo at our local children's hospital. I called at 10:30 and they gave me a 12:30 appointment! She did great for that. When I told her she had to have the echo to look at her heart Harlie said, "Oh, my heart is fiiiine, Mom." That kid seriously cracks me up. Its amazing to me that she can have so much inflection in her voice with her limited movement.

The cardiologist emailed me back and explained his concerns. He also shared his note that he sent to the plastic surgeons.

I understand, truly, how difficult it is to schedule an OR case like this, both for you and your staff and for the family.  I would like to do all I can to accommodate, even with this late notice.  However, Harlie has exceedingly complicated single-ventricle heart disease further compounded by comorbidities including restrictive lung disease.  I have not seen her in 4 years, and the available outside records provide scant details.  I regret I cannot provide cardiac clearance without reviewing the data from her most recent heart catheterization (report and angiograms), obtaining a full echocardiogram here, and examining her myself.  Additional cardiac testing may also be required.  It is likely, given the underlying complexity of her disease, that I will want to discuss the findings with cardiology and cardiac anesthesia colleagues to arrive at the best possible recommendations for her perioperative management.  It will not be possible to accomplish this all on Monday.  I wish it were otherwise.

Once I read that, I totally understood where he's coming from. It would be hard to argue with his reasoning. And, like I said, I have "forgotten" (blocked it out, maybe?) how complex she is. Plus, I know how scheduling works and I get that it would take miracles to pull this off.  

He also said...
I can’t tell you how sorry I am that you have to worry about this, especially under circumstances that are already stressful as you prepare to come up for Harlie’s surgery.  I have copied below the email I sent yesterday to the maxillofacial surgery team.  In the interim, I have received the report of the 2/6/2015 catheterization performed at Children’s National.  Our clinic, echo, and pacemaker staff are scrambling to fit Harlie in for evaluation in my clinic on Monday.  I have initiated discussions today with both my cardiac ICU and cardiac anesthesia colleagues so they can be prepared to weigh in on Monday, once we have all the data.  
It is truly wonderful that Harlie is doing so well overall, and I certainly understand the concern related to her jaw.  We all want only what’s best for her.  I will look forward to seeing her on Monday and hope we can agree to move ahead as planned on Tuesday.

Isn't he great?! The bottom line is that I get it. And I am so grateful to him for keeping her best interest in mind. At this point I have to accept that someone didn't communicate all the information to all the pertinent people. And I have to shoulder some of that blame. I should have asked more about cardiology. Must make a mental note to make a bigger deal of her cardiac care, despite my wanting to forget about it.  I just hoped and prayed that the schedule could be adjusted to fit her in on Monday.

I spent all day on the phone or the computer. It was exhausting. Then at 3pm Amanda emailed me that cardiology had worked it out and she had a full cardiology work up scheduled including a chest x-ray, EKG, pacemaker check, echo and a meeting with the cardiologist. Hallelujah!!!  
Harlie getting an EKG.
We also had appointments with all the pre-op people and the other surgeon (Dr. Resnick) since he is new to us and Harlie is new to him. That appointment was at their satellite office just outside of Boston. Our plan was to uber it there.  Amanda said the team didn't want us having to do that with our marathon of a day, so they changed her appointment to meet with Dr. Padwa here at the hospital. Ahhh, thank you!

I can't begin to say how grateful we are to all of the people who worked so hard to help us get what we needed! I feel very hopeful that all of her tests will go fine with nothing alarming and that he will feel comfortable to clear her for surgery.

It is now 1:30 and she has had all her cardiac tests and we are about to see the cardiologist.

It is now 2:30 and that went great! He said that her heart cath numbers were very reassuring. Her heart cath was done after he saw her last, so that was new info for him. I'm feeling so good about myself right now! He said he had never gotten a heart cath report that fast, and he didn't know how that happened. I said that was me! I called them and told them our situation. He said he bet I could be very persuasive. Haha!

He asked me if she ever has chest pains (no, thank God) and I told him about Harlie telling me that her heart is fine. He said, "You should have just emailed me that and it would have saved us all the trouble." Haha! I do love a good sense of humor!  He ended by saying that he wasn't going to stand in our way. She does need another heart cath, but really we are at a standstill with her jaw the way it is. Gotta fix this jaw situation in order to do that.

So, we are a go!! So, so thankful!

Surgery time is 7:30am, and we have to be here at 6am. Surgery should take about 4 hours.

We are about to meet with Dr. Padwa and I think we will be done! Marathon day (woke up at 3:45am) is almost over and its been totally fine/rather enjoyable with all cooperation and no complaints from Harlie! She really is an amazing patient. 

Check out the bling on her shirt! Thanks Lindsay!

It is now 5pm and we are DONE and back at the hotel. We are looking forward to some wine and some sort of dinner tonight. Thank you so much for your continued love and support!