Heart Update

Hi. Here's another thing that's been on my mind - Harlie's heart. l think I'm just going to think out loud and hopefully it'll make some sense.

She has a Fontan (simple description - she is a single ventricle and her circulation works entirely different than a normal heart). 

She's had some NSVTs (non-sustained ventricular tachycardia) in August and October. I've tried to google this - but I think NSVT is different for patients with congenital heart disease. I don't need to know more than I need to know, so I just need Harlie's doctors to explain what it means to HER. 

She has a pacemaker. The wires that were put in her heart were put there during her very first open heart surgery when she was just four days old. That means that those wires are now over 17 years old. Apparently, that's getting old as far as pacemaker wires go. This issue has me confused. We've seen several Electrophysiologists (pacemaker docs) and I gotta tell you, I don't understand it. The next appointment we have, I'm making Tom go with us because it is impossible for me to go to an appointment that I don't understand the info - then come home and tell Tom anything useful about the appointment. 

I'm going to try to explain why I'm confused. The pacemaker has wires that go into her heart. They connect to the generator (battery) that is in her abdomen. The generator was put in when she was five years old. Oh my gosh, my memory is going bad. I can't remember how many times the generator has been replaced! Once? Twice? Ugh. Okay, I just searched my blog and found a post that I started writing about this subject and never finished! So, she had her first generator replacement surgery on May 6, 2022 (she was 15, so the first generator lasted 10 years). 

For a year to months prior to the first generator replacement, we had regular check ins and we watched the battery life decrease. When they put the magnet thing on her abdomen, it reads all the info from the generator and the battery and the computer will try and predict the battery life remaining, based on current usage. The more the pacemaker has to fire, the more battery it uses. With the first generator she had (from age 5-15) we had to call in pacemaker transmissions from our home phone. Leading up to the generator replacement was kind of stressful because they would be like 6 months left, 5 months left, 4 months left and I'm like so schedule it already! Anyway, so it was finally replaced in May of 2022. The new generator is awesome and hooks up to Harlie's tablet, so they set the transmission frequency so we don't have to do anything! That's my kind of device, haha!

During Harlie's TMJ surgery in April 2023, I remember the docs struggling with her pacemaker settings. Anytime she goes into surgery, they have to change the settings to be stronger or something for when she goes under anesthesia (also, it depends on what equipment the surgeon is going to use - some equipment can make the pacemaker do stuff the don't want it to do). After surgery, they change the settings back. While she was recovering in Boston, they asked me if I had heard anything about her wires. I was like, um, no. So, when we had her appointment with her pacemaker doc in August of 2023, I asked her if she received any clinical notes from Boston. She did and she noticed some readings that made her put a 48-hour Holter monitor on Harlie. She said that the wires are showing signs of age. I don't remember her words exactly - but the way I understood it is that she was having to adjust the way the pacemaker works because one of the wires wasn't working consistently, or something like that. That's when she told me that 17 years is getting up there in age for wires. 

She told me the signs to look for should the wires stop working. She said that Harlie would be lethargic and might not feel well and her heart rate would be less than 60 beats per minute (the pacemaker doesn't let her heart beat get below 60bpm). She said if the pacemaker were to suddenly stop working, her heart has an escape rhythm of about 30 some beats per minute (what her heart would beat on it's own - without the help of the pacemaker). So, we would be able to get her to the hospital. I wasn't told any of that when we were just looking at the battery dying. So, it sounds to me that the wires are a totally separate issue than the battery life. I mean, they can see how much battery life is predicted any time they want. So, it shouldn't run out of battery all of a sudden with no warning. 

So, when are these wires going to stop working? Replacing the wires is a bigger deal than just replacing the generator because the wires are in (or on) her heart. Of course, no one can tell me. So, one day in September, she could barely make it from the school bus to sitting in the chair in the kitchen. She didn't feel well, she looked terrible and was super tired! So, I ran and got the pulse ox and just knew that her heart rate was going to be less than 60bpm. But, it wasn't. So, then I took her temperature and she had a fever. So, I knew it wasn't a heart thing. Then we realized she had Covid. But, seriously, how is a parent supposed to live like this?! 

Then in October she had that TMJ surgery in Boston. During that hospitalization she had another run of NSVTs plus she had really low blood pressures. I can't remember if they upped her pacemaker low or if her cardiologist did it after - but around this time they changed her low setting from 60bpm to 70bpm. They also said that she needed to go on a beta blocker for the NSVTs and they wanted her to stop taking the Enalapril (she's been on that her whole life) because her blood pressures are too low. While that might not sound like much - that's three new things! 

We came home from Boston on October 14th, I believe. Then on Tuesday, October 24 - Harlie had an appointment with her local cardiologist to follow up on all her heart stuff. I really like him and was looking forward to talking with him. I just felt like he would give me his opinion on what is going on with her and he would tell me how worried I should be. Plus, I think he could help me understand this wire thing. 

When we got there she got an echo. That took about an hour. Apparently her anatomy makes imaging difficult. Plus, she has a lot of scar tissue around her heart. At any rate, the echo shows all her normal issues - no new issues, so that's good. While they were doing the echo, I emailed her cardiologist the clinical reports from Children's National and from Boston. That's when the doctor doing the echo told me that her cardiologist wasn't in town and we would be seeing a different doctor. Ugh. Seeing a new to Harlie doc versus a doc that we've known for 17 years are two totally different appointments. I wasn't prepared for a new to us doctor. Her cardiologist emailed me right back and told me why he couldn't be there and who we would be seeing instead. He told me that I would like him. 

While he was right (I liked him fine) it wasn't the same appointment I wanted it to be. There was also another person with him. She was a transition coordinator or something. The doctor said the same thing her other doctor said about what to look for and her escape rhythm, etc. Okay, so when will her pacemaker need to be replaced? No one knows. Since this wasn't a cardiologist with whom I have a good relationship I couldn't say, Cut the crap, doc and tell me! Haha! 

So, then the transition coordinator took over. She basically talked about what it looks like after kids turn 18. It is complicated (no surprise there) because for some of her medical issues, there aren't adult specialists who know congenital issues, so she will have to keep some of her doctors. But, since kids with congenital heart disease are living longer, they have developed a new specialist - an adult cardiologist who specializes in congenital heart disease. I think she told me it takes like 25 years to develop a new specialty. Anyway, I wasn't prepared to have this kind of conversation. I know she's getting older and that's great and all, but change is SO hard for parents who have children with chronic health issues. When we find people we like and trust - and they like and trust us, it is so incredibly hard to lose them - or have to start all over with new people who don't know us at all. So, listening to her and starting to think about all the changes that might happen - well, I started to cry - and I hate to cry. Especially in front of someone I just met! I mean, I have a reputation to keep - I can't be crying at appointments! Then she told me that she makes someone cry once a week - or some kind of lie to make me feel better.  

They also told me that Harlie has to start seeing a Hepatologist (liver doctor). They don't have pediatric hepatologists because kids don't have chronic liver disease, I guess. But, with kids with Fontans, they know that a certain percentage of them can develop liver disease. Here's what I read:

One of the hallmarks of Fontan circulation is permanently elevated central venous pressure, which leads to congestive hepatopathy. Subsequently, liver fibrosis, cirrhosis or hepatocellular carcinoma may occur, all of them constituting an entity called Fontan-associated liver disease (FALD).

Since Harlie is 17, it is time that she started to be watched for this. It sounded like this was the normal process for kids like her, so they would schedule an appointment for us. 

I am especially worried about Harlie, because she is not just a Fontan. She also has lung disease and her terrible airway. For many years she had very high Fontan pressures. She's been on Sildenafil (viagra) for four or so years I think. That has helped and definitely brought her pressures down. But, clearly she's had way more years with higher pressures than with lower pressures. So, what does that mean? Is there a scale of how bad her liver is? Are there signs? I'm told there is no treatment or ability to stop it from happening. I guess if a patient has a good heart, but their liver gets bad, they can get a liver transplant. I really don't know much at all. 

I don't remember how I was notified, but months ago, I was told that Harlie had an appoint with Hepatology on January 30. I wasn't feeling well, but obviously, I had no choice but to go - I mean we've waited three months for this appointment. I wanted Tom to go with us since this was new information and I didn't know what I was going to learn. But, his work schedule didn't allow it. The day before the appointment, I get a call to check in. She said she was checking Harlie in for her GI appointment. Confused, I said, no, this isn't supposed to be a GI appointment, it is supposed to be for hepatology. Also, we already have a GI person, and this was not our GI person. So, I said does she handle hepatology under the GI department? She said, yes, she does. I looked and Gastroenterology, Hepatology and Nutrition are lumped together on VCU's website. So, it kinda made sense that the scheduler said GI appointment. Okay, fine. 

So, the next morning, Harlie and I head on down to VCU. We check in and are shown to the exam room. The nurse gets her vitals and then asks, "Do you have any GI concerns today?" Um, no. So, I said, "This is not supposed to be a GI appointment. This is supposed to be a hepatology appointment." 😑

We all know where this is going. 

The GI nurse practitioner comes in and immediately apologizes. There's been a mistake. Not sure how it happened, but somehow her hepatology referral got changed to a GI referral. She said something about since they don't technically have a hepatology department. Needless to say, I was ticked. Like, for real. I told her that Harlie missed a day of school, I missed a day of work and we've been waiting for this appointment for three months. UGH! Does no one care about my mental health?!?! 

I know it wasn't her fault - and I told her that as well. But, also, we already have a GI person! Ugh. She said that she was sorry and all that stuff. But, then said that she was really glad we were there because our food order for Harlie had expired and she was surprised we were still receiving her food order from the supply company. Apparently some higher being requires that patients be seen every freaking six months to get food. Are you kidding me? She's 17, done growing - so there are no changes to her food order. Absolutely no entity thinks about the special need family when it comes to care. At this point, I will never be able to work full time again (well, get paid for working full time, I mean). 

We left and I was really feeling so crappy. Literally every aspect of caring for her is harder than it should be. How is this sustainable? It isn't. This is another reason why I am feeling so burned out, overwhelmed, alone, etc. 

Plus, I didn't get to learn anything about her liver. Also, I just want to say that any comfort I might have felt that this is the normal process for kids like her - is gone. It doesn't feel like kids see hepatologists around here. This feels like it isn't normal at all. 

The next day I got a message to call and schedule an appointment with a hepatology doctor and they gave me the number. I called and after holding for 15 minutes, they told me they were going to have to call me back. Of course. They called back and told me that they were going to find a place for her but it would take some time. The next appointment was in April. Part of me wanted to say that it isn't urgent. I mean, I don't think it is anyway. But, then I was like, oh well, I guess I'll let them work us in. A few days later, they called me back and gave us an appointment for February 16th. Wow, okay. 

I have no idea what to expect. Will they draw labs? Do any other tests? Or just talk about it then set up tests later? I don't know. Sometimes stuff like this weighs so heavy in my mind. On the good days, when I feel stronger, it doesn't bother me as much. I guess the stronger I am, the deeper I can bury it. If you've ever had to dig a hole in your yard, you might be able to get it. I don't know. Today, as I'm writing this, it feels pretty heavy. Like, my stomach hurts as I'm thinking about it. So, I'm going to take a break. Harlie is home sick again today, so I'm going to go do some stuff with her, then I'll come back and finish. 

A few days later....

Today is Friday, February 9th. It has been a few days since I started this post. Today, I am really struggling. I think I'm going to start a new post, since that will sort of be a new topic. I'm trying really hard to keep my posts a little more focused.

Harlie has missed another three days of school this week. I'm not sure what that's about. But, she has picked up her guitar a lot lately, so that's cool. 

Also, here's a Facebook memory from a few years back. 

Here was the other picture.

I forgot to mention in my TMJ post that she appears to have more facial paralysis since her TMJ surgery in October. Her smile isn't like that anymore. I'm not sure if she will regain it or not. I guess time will tell. Makes me sad right now, though. 

As always, thanks for checking in!

Much love,

Christy xo

Our trip to New York City

Hi!  Sorry for another long delay in posts.  This one has taken a while to write.  I just don't get the alone time needed to focus.  Anyway, after many attempts, here are all the details about our trip to New York for the Christmas tree lighting at Rockefeller Center.

We were able to bring Harlie home from the hospital on Sunday, December 1st.

I can't remember if I mentioned it in one of my blog posts when she was in the PICU or not, but she was holding on to fluid.  I noticed that her legs looked swollen.  I told them at rounds so they weighed her and her weight was 69 (five pounds more than when she arrived).  The doctor came and examined her and she had pitting edema in her legs. So they gave her some Lasix (a diuretic) to help her get rid of the extra fluid.  Holding on to fluid is really hard on her body (especially her lungs). We went home with a script so we could give it to her if needed.  We had to weigh her each morning to monitor it.  She was 65 pounds on Monday and 66 on Tuesday (she was 64 at baseline). This is a girl who has needed calorie boosters her whole life to try and gain any weight at all.  Also, keep in mind that she is tube fed a formula - the same amount every day.  There is no cake, no ice cream, no variation in daily calories to explain any weight changes.  Fast weight gain is definitely fluid and needs to be resolved as quickly as possible.

We went to see her pediatrician on Tuesday, December 3.  She mentioned that Harlie's belly looked big.  This was an issue in the hospital as well.  Her pediatrician wanted me to take Harlie to her GI doc to rule out Ascites (another fluid issue).  Keep in mind that this was Tuesday.  And Tom and I were leaving the next morning to fly to New York.  We were due to fly back home Friday night.  So, I wouldn't be able to take her until the next week.  And there was NO way this could wait that long.

So, I emailed her GI doc, brought her up to speed and asked when she could see Harlie.  Tom and I left the next morning for New York.  I would never do this under normal circumstances.  But, I had no control of the schedule and this was a once in a lifetime opportunity.  Harlie's nurses assured me that they would handle whatever came up and they told us to try and have fun and relax.

Wednesday, December 4

Mona met us at the airport in New York Wednesday morning.  We shared an Uber to our hotel to get us checked in.  While in the Uber, I got a call from our nursing supervisor that something happened with Harlie's Medicaid and home health nursing approval - it was denied (a paperwork mistake).  Keep in mind that we were using our home health nurses 24/7.  So, our nursing agency had to file an emergency appeal.  And she needed my signature to do so.  We tried to handle it electronically since I had no access to a fax machine at the time.

It was too early to check in at the hotel, so we dropped off our bags and we made a plan to meet Mona and the other veterans later that afternoon.  We went to grab some food and walk around a bit.  We walked to Central Park and walked around a little. 

Then went to a bar and got some fancy drinks.  This was Tom's smoky Old Fashioned.

During this time, I was emailing her GI doc and her nurse at home trying to work on getting Harlie seen.  Her GI doc wanted labs done, so Caylee took her for labs that afternoon.  We scheduled a time on Thursday for her to see her GI doc for x-rays/ultrasound.  I was definitely worried. Harlie's weight that morning was 68 pounds - up two pounds from the day before, and up four pounds from her baseline (this was while she was on Lasix!).  I was also in contact with Harlie's cardiac social worker at CNMC (Children's National Medical Center in DC).  The simplest explanation is that when a patient has a Fontan physiology (the way Harlie's heart works) and they have a hard time processing fluid - that is a very bad sign (and could mean her Fontan was failing, which is horrible).

So, just for a minute, I want to explain something.  We know her Fontan will fail one day.  Her heart, the way it is today, is not cut out to last her till she's old and gray.  We know hard times are head of us when it comes to her heart.  So, when this fluid issue came up... well, I can't really explain it.  I guess since we know what we know, I expected to be able to handle it differently - better, even.  I was shocked at how incredibly fearful I was. It was hard to breathe. I had to make a concerted effort to take a deep breath. I guess this proves that there are some things you just can't prepare for.  I wanted to rush back to the airport, jump on the first plane to Richmond and go home to get her wherever she needed to be to help her immediately.  To be in New York and not be able to have my eyes on her, was so incredibly difficult.  I didn't even want to tell Tom.  But, as I was sitting there, trying to look fine, I realized I couldn't be this scared without him.  So, I told him.  He is so good and we got through the moment together.  There was nothing we could do at the time.  We really didn't have that much information yet.  She was scheduled to see the GI doc the next day at noon.  We needed to wait and see how that went and see what her labs showed.  We couldn't do anything until all that happened.  So, flying back that night wouldn't have changed a thing (other than my comfort).

We had to shake it off and go back to the hotel to check in and shower and get ready to head to Rockefeller Center.  We had to meet all the others at 4:30 in our hotel lobby.  On our way back, we turned the corner and saw this...

We looked at each other and thought, how crazy, we need some hope right now! And for those of you that know me best, you know how I feel about hope. Hope is a funny thing. Sometimes it just comes out of nowhere.   

While on the way to Rockefeller Center, we quickly realized that having Harlie with us would have been an enormous challenge.  There were so many people.  And they had some roads closed around the center, so in order to cross the street, we had to go back, go down the stairs to the subway, then back up the stairs to end up on the other side of the street.  All we could think about was how difficult that would have been with Harlie in her wheelchair and tethered to the oxygen concentrator.  It would have taken us SO much longer and so much more effort.  Not to mention that she was in no condition to be with us anyway.  While we were feeling what we were feeling and walking there, we were with another family of five.  Three healthy kids, all able to walk on their own and keep up with the adults.  Oh, how different our life is and how many triggers we see every day that remind us of that.

Then we went into the the Tonight Show with Jimmy Fallon entrance and we waited for us to be checked in there.

We went through a security process, which took a while and then we were escorted to a conference room upstairs.  We had to wait there for several hours until it was time to go outside.

I guess it was close to 8pm when we were escorted downstairs.  Again, we had to go through security and that was a little bit of an issue because one of the guards told our escort "no."  She was stunned and said, "but they have a spot on TV, they have to be there."  He didn't seem to care.  So, she had to go and get someone else who flashed his badge at the guy and got us through.

The stage for the show was right under the tree and the crowd that had some sort of "in" was standing on the ice rink (they put carpet down for us to stand on).  We were so close it was crazy.

We stood and watched the show for probably an hour or so until a guy came and got us and moved us to the front row, in front of a camera. Like right in front of a camera.

There were 10 of us in the spot on TV.  I can't remember if I explained it or not, but it was a veteran thing.  Toyota aired a commercial about soldiers coming home and then after that, they went to Hoda Kotb who said something like, Thank you to Toyota for supporting our veterans. Then Al Roker said something like, We are so happy to have some military families with us tonight. Then they flashed to the 10 of us standing there.  They wanted two families and it would have been awesome to have our kids with us.  This is one of those times when my feelings are so conflicted.  I'm grateful for the experience.  But, I really wish our life afforded us the ability to take our kids with us whenever and where ever we wanted to.

A while after that was the countdown to the tree lighting.  Seeing the tree at all during the season has been on my bucket list.  I just can't believe we were there for the lighting!  It was awesome and we loved it.  After that we all said good-bye and Tom and I went out to find a late dinner and some drinks.

Thursday, December 5

We had a hard time sleeping that night.  Harlie was very heavy on my mind.  It took so much effort to try to keep my fears at bay.  Tom let me be lazy and he ran to get some coffee and bagels (a true splurge food in our book).  Then we showered and headed to One World Trade Center and the 9/11 Memorial.  Tom really wanted to get that in, just in case we had to leave later that day.

They have the names of all those that died around the perimeter of the memorial pools.  Each year, on their birthday, they put a rose on that person's name.  I found this really moving.  December 5th is Catherine Lisa Loguidice's birthday.  She was 30 years old.

I was already feeling pretty weak as far as my emotions went, so we elected not to go into the 9/11 museum.  I really didn't think I could handle it.  I found the rose on Catherine's name hard enough.  So, we went up the Freedom Tower to the observatory. The elevator ride up is very cool.  It goes up 102 stories in 47 seconds.

The view from up there is amazing.  And we lucked out with a clear day.  We took our time walking around and looking at every possible view.  After a while, we decided we were done and it was time to head back down.  Right as we were passing the bar/restaurant area, they opened it.  Tom looked at me and said, "Bloody Mary?"  Um, yes! So, we splurged again with a drink with that amazing view - something we never get to do.  Thank you to our dear friends for the Visa gift card - because those expensive drinks were on you!

Then, right before noon, while we were enjoying our drinks, Brandy (Harlie's nurse) called me.  She had already seen her GI doc (for a noon appointment!) and she said she was FINE!!!  Her labs looked good and she did NOT have Ascites and all signs indicated her Fontan wasn't failing.  She said her body probably just got behind and was working so hard for several weeks - so she just needed some extra help getting the fluid off for a little while.

I wanted to cry I was so incredibly relieved!  I am so glad we stopped for that drink because we had something to celebrate!   This was the highlight of the trip for me.  Sitting and facing the windows and seeing the skyline and enjoying this downtime with Tom was really amazing.  We were so grateful!  Still are.

After that, we left and headed to Greenwich Village.  We found a cute little barber shop and I made Tom go in and get his beard trimmed.  He was being especially nice to me considering the circumstances, so I took advantage.  Haha!

Then we went to a meatball restaurant.

While this isn't a great photo - doesn't he look so much better/his age with a trimmer beard?!

The food was great and the drinks were even better...

While there I got a call from the nursing supervisor.  She said my electronic signature for the home health nursing appeal we tried yesterday didn't work.  She said I needed to write a letter.  So, I took our receipt, turned it over and wrote it on that.  I took a photo of it and sent it to her, crossed my fingers, and we went on with our day.

Then we headed to High Line park.  That was awesome.

After we were done there we stopped at a cute little coffee shop (not Starbucks) and got some Spanish Lattes.  They were delicious.

Then we headed towards the Empire State Building.  That was on my list of things I wanted to do.  But, once there we learned that the tickets were like $76 a piece!  So, we didn't go up.

By this time we had walked probably about 8 miles and were getting a bit tired.  So, we stopped at a cute bar and had another drink - gotta stay hydrated!  Haha!  Then we headed back to the hotel to shower and get ready for going out.

Crazy coincidence, our friend and neighbor (there are two houses between us) was in Manhattan for work.  So, we met her at a bar and hung out for a few hours before she had to head to the airport to fly back home.

After we left there, we headed back to Rockefeller Center.  I wanted to see the tree one last time - while we were in a much better mood.

Then we walked around Times Square.  I think we walked well over 10 miles that day.  We had a late dinner and called it a night.

Friday, December 6

We got up and had bagels again.  Sorry, couldn't help it.  They were really good and we were in New York, after all.  Don't laugh, but I wanted to see the tree one last time - during the day.  Haha!  Tom was such a good sport and didn't argue.  Plus, I wanted to go to Macy's, too.  I was trying to soak in the Christmas spirit after some hard weeks back at home.  I had no idea that Macy's was 8 floors!  That place is huge!  And the wooden escalators!  Wow!  We rode them all the way down from the 8th floor.  I wanted to go into FAO Schwarz, but the line to get in was insane, so we kept walking.

We walked to Hell's Kitchen and got some ramen at Ivan Ramen.  Tom and I had watched Chef's Table on Netflix with him (so good, we highly recommend this episode) so clearly we wanted to go.  It was delicious.

We stopped for one last drink on the way back to the hotel to get our luggage, then we headed to the airport.  It was a whirlwind trip and we are so grateful we got to go and experience it.  Thank you to all who made it possible, especially our dear friend, Mona!!

Thanks for reading!
Much love,
Christy xo

Cardiology Appointment

Harlie had a bit of a rough day today.  I picked her up from school around 12:45 and went to her speech therapy appointment at 1pm.  After that, we went to her cardiology appointment with one of my favorite doctors - Dr. Gullquist.  Here's the skinny on my concerns:

Higher oxygen saturation levels:  Her sats are higher now than ever before, which is wonderful!  She's lived in some pretty low numbers (60s and 70s) and is now living in the high 80s and low 90s.  In the many conversations I've had with her doctors, this usually meant that if her numbers were higher, that meant that they could close her fenestration.

I will simplify the explanation to this:  during her last heart surgery (called the Fontan) they created a hole (fenestration) in the connection that carries the blood from her body to her lungs for the blood to escape during higher pressures.  If her sats were low, that meant that her pressures were high enough that the blood had to escape through the hole.  As the pressures decrease, the blood passes the hole and does not need to escape, causing better oxygen saturation levels.  I think the normal thought was that then you close the hole and all is well.

However, it is not that easy.  Here is what I understand... leaving the fenestration open (as it is now) carries a low risk of the patient having a stroke.  Performing the actual procedure of closing the fenestration and for the next six months after - carries an even higher risk of the patient having a stroke.  And, because of that, they have to really thin the blood even more than it is now.

Plus, they think that in a failing Fontan (which is what will eventually happen, requiring a heart transplant) that if the fenestration is closed the patient gets sicker, faster.  And if left open, the patient essentially buys a little more time to get a heart transplant.  I'm not saying that if closed they can't get a transplant, but I think the window of opportunity is shortened.

The reasons to close the fenestration are:  1) if the patient wants to exercise more.  So, if she wanted to run, for example, her sats would go down pretty quickly.  The heart and lungs just can't keep up with the oxygen demands, requiring rest times.  And 2) I can't really remember.  Maybe it was to raise the sats a little?  But, I told him that I saw 94 on her monitor the other day and he said that's about as good as they are going to get - even if we close her fenestration.

So, I really don't see any reason to close it.  I'd rather go with the smaller chances of a stroke and a bigger window of opportunity to get her a new heart one day.  I suppose if she ever does want to run or exercise we can revisit the situation then.  Things are always changing and developing in the medical field, so who knows what they will learn in the next 5 to 10 years that might change our decision.  And things are always changing with Harlie, too.  So, we'll revisit this later if need be.

Low heart rate at night:  Over the years her heart rate has been dipping lower and lower at night.  I haven't really been that concerned because I know she has second degree heart block, so her heart doesn't beat at a normal rhythm - it will have longer pauses between beats on occasion, which makes the monitor indicate a lower beats per minute number.

This was expected to happen eventually, which is why her surgeon placed pacemaker leads in/around her heart during her first heart surgery at just four days old.  So, she's sporting a Holter monitor for the night to see what's going on.  I am pretty sure that she won't dip down to her lower heart rates tonight, just so she can make me look like an idiot.

However, if it is true that she is dipping down to lower heart rates than desirable, we will need to go on ahead and get her pacemaker hooked up and working.  Everything is ready to go, they just need to install the battery device in her abdomen area and hook it up to the wires.  I'll discuss the ins and outs of that when the time comes.

Bony protrusion to the right of her sternum:  I recently noticed that she has a bony growth just to the right of her sternum.  Since they cut the sternum for open heart surgery and then use wires to put it back together, the bone can just heal over the wires like that.  I knew that this could happen.  But, I really thought we were in the clear.  It's been a year and a half since her last surgery after all.   So, we do nothing for a long time until we think it needs to be fixed.  Then they can shave the bone down.

When I noticed it, I automatically assumed that's what it was and so I wasn't worried.  But, then today someone made me wonder if it was something more.  Or different.  Then I thought, "what if?"  And then I thought, "what if I miss something big one day because my perspective of what's important is so skewed now?"  Eh, that's just a bony sternum, not a mass of something deadly.  Eh, that's just a screw coming out of her jaw, no biggie.  Oh, her sats are 70?  Whatever, they've been worse, I'll just give her some oxygen. Eh, her heart rate is 35?  Whatev, I'll just lower the alarm setting so it doesn't wake me in the night. 

I will say that I decided I will have to get CPR certified soon.  Couldn't hurt.

She had an echo done (ultrasound of the heart) to check things out.  She has a mild leak in there.  It's still there, and still mild today.  So, that's good.  I don't ask anything about it, really.  Because I'll deal with that problem should it ever arise.  That's what yearly check ups are for, right?  So, you know how they do an ultrasound with the wand (or whatever they call it) and the gel?  Well, it doesn't hurt.  But, try telling Harlie that!  WHEW!  She HATES getting an echo done.  I tried to reason with her, but she would have none of that.  I finally had to just hold her hands.  I did manage to get her to hold my phone so she could play Angry Birds or something.  Holding the phone did get her to calm down a little for a bit, but she wouldn't play it.

So, then Beverly (who did the echo) had to put the Holter monitor on her.  Oh boy.  That was torture.  It's just a bunch of leads stuck to her chest.  With wires attached to the leads.  And then taped to her skin. What's the big deal?   The wires are plugged into a reader and she wears the reader around her neck/shoulder.  Oh, did she cry!  After the monitor was in place, I tried to put her dress back on.  She didn't want any part of that.  If the dress went over the monitor, then that meant she had to leave with it on.  And she was not happy about that!  It was a long struggle to get the dress on her.  I finally bribed her with the promise a Curious George DVD in the car and movies at home.

Once the dress was on, and the monitor went over her shoulder, she has not let it go.

I think she's afraid someone will tug on the wires or something, so she's keeping it close to her.  Although I did manage to get a smile out of her...

When it was time to go to bed, she would NOT - I repeat NOT - let us take off her dress.  So, she's sleeping in it.

The monitor can come off in the morning.  I am so glad she doesn't have to wear it to school.  But, I am not looking forward to removing it!  I will have to see if I can get Terri to do the dirty work for me.  I'm pretty sure she's going to be late to school.

Oh, and I just had to go upstairs and lower the alarm setting on her pulse ox.  We set it to alarm at 40 or below.  And it alarmed enough times that I had to change it to alarm at 35 or lower.  So, maybe it will be indicative of what's been going on after all.  I have to run the Holter monitor back to MCV tomorrow and he said he'll let me know the results early next week.

So, that's it for tonight.  I have way more to blog about and I'm really hoping I can do that this weekend. Brandy is coming over this weekend to help out, so I think I'm going to skip over to the library to get some peace and quiet with my computer during the DAY so I don't have to miss out on sleep.

Thanks!
~Christy

Good News!

We are going home!!! 

They took her pacer wires out and she has to stay for observation until 5:30pm, and then we are FREE TO GO!!!

Harlie is SO over this hospital stay.  She is fighting everything - even suctioning, something she deals with all the time, all of a sudden is too much.  I think she is just trying to regain some control over her life.  Who could blame her? 

And she's had another rough day.  They removed her chest tube dressings and removed the sutures.  They said that the spots heal better without the sutures in there (not dissolvable ones).  And one of hers was already looking angry and red.  But, in just starting to remove the dressing, the nurse practitioner had to call in for reinforcements.  Harlie was fighting - and HARD! 

After that she took out the pacer wires.  Earlier she had the Holter monitor removed.  All the stickers everywhere have thoroughly pissed her off!!!  She hates those things! 

Then came the removing of her IV.  They had so much tape around it, that was agony!  But, once it was out, she felt so much better. 

Last night she made me a bit nervous (only in that I thought maybe our discharge today could have been threatened).  Her nurse didn't like her high blood pressures and her high respiratory rate.  So, she called the resident and after going over some things she guessed that she was probably just in pain.  Which really made total sense.  Duh.  So, they gave her some Tylenol and took her blood pressure a little while later, and it was fine.  Whew! 

I think she realizes she's going home.  Tom took the train up this morning and checked us out of the Ronald McDonald House.  When he came back up to her room she saw the stroller.  We haven't gotten her dressed yet, but that is going to happen as soon as I get back to her room after finishing this post.

I will have to go into more detail later, but the Holter monitor showed that she is staying in Junctional Rythm.  They said that right now it is fine and that she could possibly stay that way from here on out.  Or it could resolve itself in time.  Or her heart rate could start to slow down and then action would have to be taken.  I think a pacemaker would have to be hooked up if that's the case, but don't hold me to that.  This is new to her.  It began after surgery.  So, we will follow-up with her home cardiologist in the next few weeks and do another 24 hours on the Holter monitor.  Then I think she will have to go back for regular tests and exams until they determine what (if anything) needs to be done. 

Wow.  Post-op day 9!  I can't even begin to explain how I'm feeling right now.  The thought of going home and having the Fontan behind us is surreal.  And it brings tears to my eyes.  It is a relief I just can't put into words.  We made it and she is amazing. 

Okay, gotta go home!!!!  I can't wait to see her mood change!

Thank you again for all your wonderful prayers, comments, and support!  Being up here can feel so isolating.  Your comments and messages are so wonderful to receive.  Please know I am so, so grateful!!!

Much love,
Christy

Post-Op Day 1

Sorry I haven't been able to update all day.  I hope you believe in "no news is good news."  Getting to a computer lately has been a true challenge.  I hate to knock on the hospital (it has many good points that we love) but it is a little slow on the wireless uptake. 

When we were waiting during surgery we were told that we would have internet connection on this floor - in the CICU and the HKU (heart and kidney unit - once she steps down to the floor).  But, that isn't exactly true.  Upon further investigation - there IS wireless.  However, there are only a limited number of people allowed on it at a time (what!?!?!?!?) and it only reaches so many feet from the nurses station.  And guess what?  Yep, you got it.  We are just outside that range. 

I mean, what gives?  How hard is it to provide wireless to more than a few people within 20 feet?  Technically, is that even still considered wireless? 

So, in order to update you (my family and loved ones) during a stressful time in my life, I have to use a computer provided by the hospital.  There are several in the library (open 8-5) and three in the family waiting room outside the CICU, PICU and HKU (that's a lot of beds, in case you're wondering).  And they offer three whopping computers - one of which isn't working.

As far as the library is concerned, that's where I've used the computer every time since Harlie's birth.  I have never had a problem getting on a computer.  Until this stay.  I couldn't get on one for hours yesterday (from either location) or this morning.  And it is a far walk to get there - so it gets pretty darn frustrating real quick.  I asked the librarian if there were less computers than there used to be.  Yes, they used to have 25 (I think).  She said that they took a survery and that the most that were being used at the same time was 9.  So, they downsized to 7 computers.  ????  She said that they are getting new ones any day now and there should be a total of 12. 

I wish they would get wireless like most other hospitals - that way I could stay with Harlie while I update, which would be wonderful.  And way less stressful.  Plus, I could upload photos.  So, I'll have to upload the photos when we get back to the Ronald McDonald House.  But, she looks good.

Anyway, enough of that...

Harlie is doing well.  At around 2pm ENT came by and removed the intubation tube from her trach stoma and put in a regular trach.  She looks normal again.  And the nurse also pulled the tube from her mouth (that went into her belly).  Overall, she looks very comfortable and is resting peacefully. 

They are putting her on CPAP now (which means she'll be doing more breathing on her own versus the machine forcing her to breathe).  Harlie definitely likes to breathe on her own and she always does better and is happier without the vent.  Plus, the positive pressure is actually bad for her circulation and heart issues.   So, we're very lucky that she doesn't need the vent for very long.

They are reducing her sedation meds so that she can start to wake up.  We'll see how that goes.  She's had a fever today, but that is totally normal post-op.  She's also starting to swell (most obvious in her hands and feet).  That is also totally normal and expected post-op.  Balancing fluid is an issue and a challenge after the Fontan.  I was told that post-op days 2 and 3 are usually the worst of it, though. 

She had another EKG this afternoon and there was some intermittent heart block (but nothing to be worried about at this time).  I think that's happened to her after every heart surgery so far.  It just takes a while for her body to adjust to the changes.  I think they decided to leave the external pacers on for now. 

So, that's pretty much it.  Not much to report on, really.  It has been a quiet day, which is great. 

As usual, thank you so much for all your thoughts and prayers!  Clearly, they were heard!  Things are going way more smoothly than I was anticipating. 

Much love,
Christy

Fontan Pre-Op Day

Pre-op today went well, I think.

She weighed in at 30 pounds and I think she measured 36.5 inches tall.  The first thing we had to do was get an EKG.  The nurse brought us a hospital gown and that's when Harlie got upset.

That was over fairly quickly, though.  And then came the torture of peeling off all the stickers.  They say it doesn't hurt that bad, but you would never think that based on her reactions.  She hates it.

It was very cool to be able to get urine for labs by taking her to the potty.  Last year they had to put a bag on her and that was a total nightmare.

Then they did an echocardiogram.  Her cardiologist said that there was some narrowing or something where her surgeon did the last surgery (he created another way for the blood to leave the heart).  But she said that it's mild right now and they would leave it alone and not try to do anything during the Fontan.  The risks outweigh the benefits.  So, we'll see how that goes over time.

Then we went down to meet with the cardiac anesthesiologist.  Then after that we went for chest x-rays and blood work. Both departments were super busy. But, luckily, our nurse practitioner (NP) who gets all this set up - came and got us to go see her surgeon, Dr. Jonas.  That went well.  Nothing new, which is good.  I feel like it was less stressful than last year when we sat down to talk about the Fontan (because we thought we were getting it then).  At this point, I'm not sure if I feel better about it, or if I just have my game face on.

The risks for brain damage or death is about 3-5% for the average kiddo getting the Fontan.  For Harlie the numbers are 5-6%.  They say that those numbers are high for them, but they seem pretty low to me.  Our NP said that I am the only parent who thinks that.  So you can tell my perspective is completely off - thanks to her previous 14 surgeries and all the odds she's beaten so far.

Mainly, the risks are excessive bleeding, infection (higher for her because of her trach stoma being so close to the incision), and pleural effusions.

We gave consent for the surgery and headed back downstairs for chest x-rays and blood work.  We chose to do the x-rays first, so we could leave the hospital the second they finished the blood work.  That way Harlie wouldn't be upset for the x-rays and we could somewhat reward her for the blood work by getting the heck out of there.

When she went into the x-ray room, she went straight for the chair where you sit for the x-rays.  She got right in with no complaint.  And when the x-ray tech asked her to raise her hands for the x-ray, she did exactly what she was told.  Then she got down and went on about her day.

Then we went for blood work.  That was a nightmare, as usual.  She sat on my lap in the chair and did so without a fight.  But when the tech got out the syringes and put on her gloves, Harlie put both of her hands behind her back.  The tech asked her if she was hiding from her.  It kills me to think about all the knowledge that girl has about unpleasant stuff.

They wanted to get three vials of blood, but they only got one and a half.  Her blood was so thick (since she's been off aspirin for five days in prep for the surgery) and they (the lab tech had to call for help) had to stick her several times.  It really is parental torture to have to hold her down to let them hurt her.  We just keep telling her that we are so sorry and that we love her.

After that was over we bolted to the car to go find something fun to do to reward her for a long, tiring day.  Tom said that he wished she could eat by mouth so he could give her whatever treat she wanted.  Instead we found a playground.

Okay, this picture doesn't do it justice, but there were these squirrels...

that are black.  And shiny.  And their fur looks softer and thicker than a normal squirrel's.  Weird.

Tom was playing around with the color accent setting on the camera and it came out like this...

In this picture you can see a little of my pink hair.  There's more under the top layer.

The playground also had a fountain area for the kids to run around in.  It had just rained when we got there, so we had the place to ourselves.

This was my view walking back to the car.  Is there anything more sweet than a daddy carrying his little girl?

There are some things that Harlie does that makes me so thankful.  Tonight at dinner she was playing with her Mickey Mouse and she did this...

Totally normal, I know.  But I don't take anything with her for granted.  Earlier, when we were leaving the Ronald McDonald House, she put Mickey on the slide, counted to three and pushed him down.  Make my heart melt!

After dinner we went to Target to get some Pedialyte for her overnight hydration (this is when tube feeding comes in pretty handy).  We were just walking around and just happened to go by the toy section.  She went crazy over all the Toy Story 3 toys.  But we left and she kept signing "dinosaur" (Rex from the movie) and pointing behind us, which told me that she wanted to go back to the toys.  So we did and Tom put her down and let her walk around and look at them more closely.

After looking around she went and grabbed a box with Jessie and Bullseye in it and threw it in the cart.  Oh, there goes my heart again.  Another totally normal thing that she's NEVER done before.  She never asks for anything - other than an activity (like watch a movie, go outside, etc.).  So, of course we bought it for her.  And, let me tell you something - if we were rich I'd buy her anything she wanted after a day like today and knowing what lies ahead.

We have to be at back at the hospital at 7:30am and her surgery is scheduled for 9am.  Her surgeon said that it took the anesthesia team two hours (which is very long) to prep her for surgery last year.  So, we are guessing that will be the case again this time.  Then the surgery will take about three to four hours.

I will, of course, update as I can to let you know what's going on.

Thank you so much for all your thoughts and prayers.  You have no idea how much that helps during times like these.  We are so very appreciative!
~Christy