Surgical Consult

On Tuesday (June 30), we had a meeting with one of the surgeons that will be doing Harlie's surgery on Wednesday. As of this writing, I'm not even sure I'm going to post this one. I am just writing to help myself process. 

Harlie's EPs (electrophysiologists/pacemaker docs) want the surgeons to replace:

1. Her pacemaker generator (located in her abdomen)

2. Her ventricular leads (they are 19 years old and are "failing")

3. Her atrial leads (they are 14/15 years old and appear to be working fine). 

The generator and the ventricular leads must be done now. However, since they are going to all the trouble to have this surgery (and there is a lot of effort) they are thinking she might as well replace the atrial leads because they are bound to fail at some point in the future. What kind of time, I have no idea. 

Here are the basic challenges in Harlie's case:

• Her heart anatomy is unique and stuff is not where it is supposed to be.
• Access to veins, etc. for life saving reasons is limited or difficult (maybe even impossible).
• Her heart is adhered to her chest wall/underside of her sternum.
• Scar tissue can easily tear and bleed

I'm going to break it down into several potential outcomes. 

Outcome A: The surgeons can successfully replace all three things - generator, ventricular leads and atrial leads. 

Outcome B: The surgeons replace #1, the generator and #2, the ventricular leads. 

Outcome C: The surgeons can only replace #1, the generator. 

Outcome A is the best possible outcome. Unfortunately, this is the most unlikely outcome. Explaining why is difficult. Mainly because understanding it myself is difficult. For one, the surgeon's first language is not English. She spoke very well, but there were times where it was very difficult to understand the subject material. It doesn't help that this is complex material.

In order to do #3, replace her atrial leads, they have to do a full sternotomy. That is when they cut the sternum to open the chest. However, Harlie's heart is adhered to her chest wall/underside of her sternum with scar tissue. It is my understanding (at this time) that in a typical open chest situation - they would cut the sternum, then put the patient on heart bypass (a machine that does the work of the heart so the heart can be operated on). 

But, that is too risky with her heart being stuck to the underside of her sternum (because they could cut her heart). I do not know if that means the answer is to put the patient on heart bypass first. That way, if they cut the heart, it is less life threatening because she is already on the bypass machine? Let's just go with that for now. 

However, it sounded like it will be more difficult to get Harlie on the bypass machine because of limited access. This was another part that was hard to understand. Harlie's femoral veins are scarred down and cannot be accessed. When Harlie had her first four open heart surgeries, that wasn't the case. They became scarred down years later after several more heart caths. I know they have gotten access through her neck (for heart caths), but I don't know if that is enough for bypass. 

So, I believe she said she does not want to have to put her on bypass (but I could be wrong about this, since this was the part that was difficult to understand). Oh, also she said that because of the adhesion to her chest wall, any small manipulation can cause the scar tissue to tear and then bleed. This can become a major problem and it is unpredictable. 

So, I believe she said she does not want to do a full sternotomy. She wants to cut the lower part of her sternum (or right below her sternum) then make a cut over to the left (creating an L shape/flap) and she wants to see what she can see/do from that area. She said she will not do more than she is comfortable doing. Keep in mind that we only spoke to one of the two surgeons. The other surgeon is the chief of surgery, but we haven't met him yet. The surgeon we met with said she trained under Dr. Jonas (the surgeon who did all of Harlie's heart surgeries and has retired). 

Every time she has had heart surgery, the surgeons have given us a risk percentage. There is no way I could find it now, but I remember Dr. Jonas writing it down on a piece of paper that he had drawn her heart on with his surgical plan. He said her risk was higher than normal and I believe it was 5-7% or something like that. But, I'm not entirely sure. I think this is the risk of having major complications or death as a result of the surgery. 

Anyway, her risk percentage this time is way higher than normal - 15-20%! At first, these numbers didn't seem very large to me. Honestly, I was like, okay, fine. But, as I am learning (and Googling), when it comes to heart surgery - anything over 15% is extremely risky. In fact, she even said, the easiest/safest thing would be to just replace the generator. While I understand that from a her perspective, that would not be the best thing for Harlie's longevity. I mean, her heart won't beat if her ventricular leads stop working. So, I felt the need to tell the surgeon that it is difficult to live every single day afraid that one of the components of her pacemaker might fail and that we would lose her.  

At this point, we still have questions. 

Like, what does it look like (for the future) if she can only replace the generator and ventricular leads?

What does it look like if she can only replace the generator?

Sadly, I don't know that they know the answers. We asked her, but she said that she didn't know and those are questions for her EP doc. So, during our meeting, she texted Harlie's EP and he told her that he would call us the next day (Wednesday). She said we would speak again on Tuesday sometime during her pre-op appointments. This was a zoom meeting and Tom was at his work and I was home with Harlie. Harlie was in the room with me during the entire call. I always make the information available to her if she wants to be a part of her appointments. She was on her tablet and her tablet bluetooths to her hearing aid, so I'm guessing she didn't hear anything. Anyway, after we ended the call, I sat in complete shock and slowly closed my laptop. Then Harlie came up behind me (she paid enough attention to know I was done) and in her sweet little voice she said, "So, how'd it go?" So, I looked at her and lied. I said, "It went fine, sweetheart." 

Then, that night I had that documentary screening and panel Q&A after. Oh, my brain was having a hard time. That took some kind of energy I cannot describe. Needless to say, I'm glad that is over. As I've eluded to before (I might have even said it outright) sharing the most painful parts of your life is really difficult. It means you make yourself vulnerable and that is terrifying. Will people handle this vulnerability with care? By sharing the documentary - that means people are either going to watch it - or they aren't. And that is scary by itself. Is seeing it important enough to someone? If you saw it, did you learn anything? Do you see me (or other parents of medically fragile children) differently? All of this to say a huge thank you to those of you who reached out and truly handled all that with care. I appreciate it more than you know. 

Anyway, back to Harlie - I knew she was hardly an easy case. But, I really did not expect there to be so many complications and so much uncertainty. Uncertainty of this level when you have 19+ years of medical trauma is a really bad combination. I am STRUGGLING. Sometimes I have to focus on just getting through minutes. Sometimes I feel such a strong need to cry. Sometimes my stomach hurts so bad. Normally, I am the one who is doing the catastrophic thinking and I'm almost making outcomes up that aren't likely at all. And I usually have Tom to tell me that and I have his positivity to lean back on or to bring me back to reality. But, I'm not doing that this time. And Tom is just as scared as I am. 

Obviously, we sort of "knew" (as much as one can predict the future) her heart could be more of a problem one day. That gets lost with all the other challenges she has. But, her heart disease qualified her to Make-A-Wish for a reason. So, even though we've known this - we are not ready to lose her. We will never be ready to lose her. 

We are trying to hope for the best. I mean, maybe everything will go great and they will be able to complete outcome A and we can put all this behind us for another few years. 

But, the back and forth - I'm afraid - it'll be fine - what if they can't - but maybe they can - I gotta do the laundry - I'm afraid - darn it, forgot about the laundry - it'll be fine - gotta walk the dogs - what if they can't - gotta go to work - but maybe they can - what would we do if... can't think of that right now - gotta get to work - it'll be fine - what if it isn't - it'll be fine, on repeat for all the hours I'm awake. Then it goes into my dreams in weird ways as dreams often are. 

So, that's where I am right now. Life is not fun right now. I'm blogging all of this because it helps me process. It helps me clearly see the holes in my understanding so I am prepared when we get in front of her doctors. I hope it helps those who know us to understand us better. I also hopes it changes your expectations - like in lowers them. Haha. I'm doing the best I can to get through this, but it comes at a cost. My brain just doesn't work like it used to. For example, we were out a few weeks ago and saw someone we knew but haven't seen in a long time. I could not remember her name. It wasn't that she wasn't important - it had nothing to do with her at all. It was that my brain is too busy surviving. 

I was in my therapy session a couple of weeks ago and she told me that when I feel danger, my thinking brain goes off line and my brain searches for safety. Of course no one can see this. I was trying to find a way to express my feelings. Like people look at me and see Christy - they think she is totally fine. She's done this so many times before - she has this! This is just another surgery and that's just what they do - no biggie. You might even talk to me and still not see what is different. So, they treat me like I'm just my normal self - the same person I've been. 

But, today's Christy has been through an especially rough few months. Today's Christy is not the same as the old Christy. Thus, I don't want to be treated as the old Christy. Basically, I want to be treated like an adorable wounded animal. When people see me - I want to hear awe, look at you, here's some water (or wine) and then I want to be helped out of the middle of the road so I don't get run over. Is that too much to ask? Haha! I will have to be really brave to post this one. I'm only writing it right now to help me right now. Although, I'm betting there are plenty of people who felt this way when they were going through a particularly rough time. So, as I have to tell myself - my feelings are appropriate for what I'm going through. I am not crazy. 

On to better things, Tom's mom came to visit this week to spend time with Harlie before her surgery. It is always so great having her here. This was the first time we have used Murphy's room as the official guest room. Tom and I got it all cleaned up and ready for her last week. We still have to paint and decorate, but I'm not sure when we'll get to that. Anyway, Murphy was off on Wednesday, so he came over and spent the day with us. We went to see the Monsters vs. Minions movie (funny and cute). Then we had dinner. The next day Murphy met Mary Ann and Harlie for lunch, then he went to work and Mary Ann took Harlie to see Super Girl and then Toy Story 5. Sadly, she had to go home on Friday. 

On Friday I took the dogs to the vet to have their nails clipped. Mabel has gotten so bad that I have to drug her before I take her. She is crazy! But, they are still so cute and funny to me. I mean, just look at them!

Well, that's it for this one.

July 8 

Overall, I think we are feeling a tad bit better after chatting with her docs yesterday during preop. I thought about just editing what i already wrote, but that was too hard. So, here goes...

Bypass issue: she explained that in order to be put on bypass you have to have a full circuit? So you need an in and an out. She does not think she can access the venous return? But, she really won't know until she gets in there. 

Lead issues: The ventricular leads must be replaced. We did not ask what it looks like if she can't replace them. I did not want to hear the answer. 

They said it won't be the end of the world if she cannot replace the atrial lines. They have been known to last a lot longer than the ventricular leads. This was great to hear! We are going to hold on to that. That means there are two positive outcomes, which is a huge improvement. 

They also explained that they can't just stick the leads anywhere. An EP will be in the OR and will be testing the leads as she places them. So, now I'm worried that there won't be enough spots available. So, I got less worried there for a minute, but now I'm back to full worried. Darn it!

Ok, I'm going to end this one now and start a new one with more about yesterday and today.

Thank you for reading, caring and loving us. We appreciate it more than you know! 

Much love,

Christy xo

June Recap

Hi,

I'll get the medical update out of the way first. After her heart cath we had that appointment with the advanced lung doctor. He walked in and after hearing her cough he said she has some kind of infection in her lungs. We were skeptical. But, they took cultures and he said that he would like her to be seen by him every three months and he'd like her to see a different doctor who focuses on lung/GI connections (I think that's what he said) every three months. Ugh. I don't want more doctors and regular appointments. This is really getting out of control. While we were there, the adult congenital cardiologist came by to meet us, which was so nice! I really wish we could consolidate her care, but everyone agrees that she really should be known in both hospitals (CNMC and VCU). So, that means she has at least two of each specialists. That's a lot of doctors. 

A few days later, the nurse practitioner called to tell me that she has something growing in her lungs. So, they put her on antibiotics. She also wants her to start a nebulizer medication called Tobramycin. I think I might have mentioned in the past that sometimes I feel like I'm being tortured. Like death by a thousand cuts. Takes a while, but eventually you'll break down. 

Well, Harlie has been on an antibiotic called Doxycycline for the past five years. Two times per day for the past five years. Well, her infectious disease doctor felt like she could come off of it about five weeks after her TMJ surgery. So, on the 8th of June, I gave her her last dose. Hallelujah! So nice not to have to use three syringes two times per day, no more mixing it, no more dealing with it, yay! Well, on the 10th she had to start this other antibiotic for her lung thing. Really?! Sometimes it is the little things that kill me. Luckily, that was for only 14 days. 

But the nebulizer meds were more of a headache. They sent us a cooler with her meds (they have to be refrigerated) and a box of syringes with needles. Huh? So, after reading it said that her dose was smaller than a single vial, so the syringes were for drawing up the correct dose. But, it really didn't seem right. The dose was so tiny and so much of a breathing treatment just goes into the air. They also didn't send me an adapter to use with a trach (they only sent a mouth piece). Anyway, after using my resources, I was able to get an adapter and I also messaged the NP to ask her to double check the dose.  She replied that the dose was incorrect and I am to give her one whole vial. She has to have two treatments per day for 28 days, then take 28 days off, then start again for 28 days, etc. 

After her heart cath, I waited a whole week before I sent a message asking about a surgery date. The waiting for information is killing me. They wrote back on the 9th that it was scheduled for June 26. So, we obviously make plans for this date. Tom schedules his work/jobs accordingly and we make no plans for the 4th of July because clearly, she'll be recovering. Then a week later, they called me to tell me that they have determined that her case requires two surgeons, so they added the chief of surgery to her case. He was not available on the 26th, so they had to reschedule it for July 8th. 

So, this means more waiting (which feels like torture), and Tom has a job starting on the 8th, which means he can't really be gone for days in a row that week. Part of me wanted to say NO! You can't do this to parents! This is now our 3rd date for this surgery. But, I don't mess with fate. The nurse said that since Harlie's heart is right behind her sternum (like closer than normal) they consider her surgery high risk and the chief wants to do it. They have to cut her sternum, but not cut her heart immediately behind it. So, hopefully July 8th is it and it won't change again. June 26th was a Friday. I didn't love her having a major surgery on a Friday. Weekends are just a different feel in a hospital and being post op day 1 and 2 over the weekend isn't my favorite. July 8th is a Wednesday. So, I actually like that day of the week better. 

To be honest, I have really been struggling these past few months. I've just not been myself. My nervous system is on edge, all the time. People have no idea what is going on in my head, running in the background, all the time. It is not helpful to be waiting for something I consider dangerous. I feel like I'm on a roller coaster going up the first steep hill, one click at a time. I'm still not at the top and it has been almost two months! I've said this before, I hate wishing time away, but I just want this behind us already! 

Enough of the serious stuff... here's the regular stuff from June:

We went to see Full Moon Fever and Three Sheets to the Wind at Maymont on June 5th with our friends Mike and Marcy. 

We went to a Flying Squirrels baseball game on June 9th.

We got Murphy's graduation photo.

The adult congenital cardiologist told Harlie that exercise would be good for her before surgery. She listened and now does a two mile loop on her bike most mornings. 

We took Harlie to Monster Golf (indoor putt putt) and it was fun. 

Cooper asked Harlie to sew a new button on his shorts so he could take them to camp with him. 

On June 17th, Cooper left with two of the Gasperini boys to drive to camp in Maine. They are all going to be counselors this summer. This will be Cooper's 10th year in a row going to this camp. This year is his first as a paid counselor. This will also be the longest he's been away since he had to go early for counselor stuff before the campers arrived. He won't be home until mid-August! 

James, Thomas and Cooper

Harlie and I gave the dogs a bath. Here is Rooney.

We went to see Lord Huron with our friends Craig and Patti at Virginia Credit Union Live - my favorite venue. 

Murphy came over on Father's Day. The four of us went to see Toy Story 5 (three of us cried). We all loved it. Then Murphy came over to smoke a cigar with Tom and then we had dinner. 

Murphy and Rooney. Rooney is 14 and definitely acts like an old man.
But I still think he is the cutest ever. Murphy is pretty cute, too. 

Just another bike ride for Harlie.

Cooper took this photo at camp and sent it to me. That is Moose Pond and it is so beautiful!

On June 24th, we went to another Flying Squirrels game. That new venue is just so nice! We went with Craig and Patti (but I neglected to get a big group photo) and Murphy met us there.

Also, last thing: 

I can't remember if I blogged about it or not, but in August of 2024, Tom and I were interviewed by my dear friend Ann for a documentary based on the book Shared Struggles. Harlie is in two chapters of the book. Well, the documentary is done and there is a free screening of it on June 30th. You can get more info here. While I feel embarrassment seeing myself on video, I do think it is a good opportunity for people to get a small glimpse into our life as parents of a medically fragile child. There are three families in the documentary and I would second everything the other families say during their interviews. It is also really cool to hear the doctors talk about their perspectives, too. There is a part in the documentary when a doctor talks about driving a trached child in a car seat. OMG, so true! Today people have videos for the backseat. Back then, if Harlie coughed, she had no airway! So I would have to pull over immediately to suction her. That meant I had to drive always thinking/preparing to be able to pull over. It was so stressful! 

Well, thanks for reading! 

Much love, 

Christy xo

Heart Cath #9

 Hi! We left Richmond at 5:45am because we wanted to make sure we were here by 9am (for a 10am start time). Since we could take the HOV lanes, we got here an hour early. 

We just hung out in the cafeteria for a bit and then headed to the cath lab a few minutes early. The nurse came out to tell us that the first case was still in progress, so our start time was pushed back to 11am. She told us to give her a little water. They don't want Fontan patients to get dehydrated.  

At some point Dr. Kanter came to chat with us.  He has done most of her caths. Turns out this was her 8th with him. But I remember that her first cath was in Richmond when she was just a few months old. After that one, she has only had them here in DC. 

Anyway, he explained everything and we felt so much better! Here's what we learned:

1. The standard practice for years has been to use a 16mm ring enforced Gore Tex for the Fontan "replumbing". 

This is the tubing they use, and
what she has in her body.

2. They are finding that size is too small as the patients get older.  To revise it, they would have to remove the 16mm tube and put in a larger one. That is a much bigger surgery and has a much longer recovery time. 

3. Dr. Kanter said they have discovered that they can put a stent in and stretch the Gore Tex to be closer to 20-23mm. 

This section has a stent in it to show you how much bigger it can get. 

This is the stent they use. 

4. So far, they have done that for about 18 patients. He said that the surgeons asked him if he could do it in Harlie's case and he said yes. Then they asked, but what if you can't? See, other people think like me, too! He said, but I know I can. 

5. He also said that he planned on closing her fenestration. This is a hole put in the Fontan tube to allow some pressure relief.  This also means that oxygen saturations go down since unoxygenated blood mixes with oxygenated blood through the hole. The practice has gone back and forth over the years on whether or not to leave it open, or close it. In Harlie's case, they have always decided to leave it open. However,  based on new practices and her current situation they decided to close it. He said if she needed it back, they could always cut another hole. He said that he believes her sats will go up to the 90s (she has been in the 80s most of her life, while most people are close to 100). He also said she will likely just feel better overall and have more energy. I really don't even know what to do with that kind of information. I mean, who wouldn't want those things for their child? WE HAVEN'T HAD THOSE THINGS FOR OUR CHILD! It seems too good to be true that she could feel better, have more energy and have sats in the 90s!! I just can't believe it. 

They took her back at 1pm!!! Poor Harlie was gagging/retching. I don't know what that was all about, since she's never done that before. She seemed to have more discomfort from being hungry than I've seen before. She didn't even say she was hungry. She kept saying she was having a weird feeling. I'm guessing it was a mixture of nerves, fear and hunger? This might be the longest she's gone without food. She's usually first case. It is funny what we take for granted with kids who can communicate all their feelings. Not only is it difficult for her to articulate her feelings, she doesn't have a normal experience/relationship with food. 

Anyway, we were all glad to end the waiting and just get on with it. After they took her back,  we practically ran outside and down the street to a bar/restaurant to eat. 

Then we walked back and waited. He said it would take him about 3-4 hours.  I guess it was a little after 5pm they called us to say he would be out soon to discuss everything. 

Here's what he said: 

1. The stent(s) were successful! However, when he was doing it (it is a whole process) he busted her Fontan. He said that while he was working the anesthesiologist said, did you just do something because her sats just went to 60. 😱 He said he knew what happened. He said she is the 2nd patient to have that happen. 

The dark area in the red circle is blood leaving the Fontan where it shouldn't be. 

He put a covered stent in to fill the gap and that solved that problem. To enlarge the whole Fontan, it takes several stents. This one was just a different kind. 

All fixed!

He said there are parts of her Fontan that is now 21mm and other parts 22-23mm. He had put a stent in a narrowing part of the Fontan back in 2017, so he couldn't make that section any bigger. 

2. He said her Fontan pressures were 16. That's really just for my record keeping. Not worth explaining. 

3. He closed the fenestration. 

4. They did the trans esophageal echo. Oh, earlier when they mentioned they were going to go through her mouth with a scope, I asked them to please check with her ENT here who knows her airway and how access is difficult through her mouth. Well, when they said access through her mouth, I immediately thought airway/ENT. Well, they are going down her esophagus - not her airway - duh!!! 🤦‍♀️ I felt like such a dummy. Like I used to be sharper than that! 

Well, when he came out he said they had a difficult time getting into her esophagus. Huh. So, maybe I'm not such a dummy! Haha! Making the turn down her airway or her esophagus is tricky because of her anatomy. 

5. They gave her a lot of fluids and heparin during the procedure. So, when it came to wrapping up, her blood clotting abilities were very slow. They have to go in through her jugular because her femoral veins are all scarred over from previous access during other caths. Plus, I think going in through different ways allows access to different parts of her heart. Anyway, he said that when he left to come see us, the person had been applying pressure to stop the bleeding for 25 minutes! So crazy. 

6. Overall, the cath was a success and he feels like things will be better for her. Tom and I came here with the mindset that things were failing and that he was going to try to put a bandaid on it. Instead, he improved things! What a great result! 

7. He suspects that the surgeons will want to do just the pacemaker surgery, with maybe some kind of valve repair. I'm not sure when we will talk to them about that. I'm guessing pretty soon, though.

Here are some Fontan numbers, just because it struck me as interesting: 

1. According to a Google search, there are about 27,000-30,000 patients living in the US with a Fontan. 

2. There are about 1,000 Fontans performed in the US per year. 

3. Dr. Jonas was at Children's National Medical Center for 15 years and performed about 180 Fontans during that time. That is 12 per year. He was at Boston Children's for like 20 years before that. 

As we were talking about things, it occurred to me how small these numbers are! Here I was thinking Fontans were everywhere and that this was like an every day thing. Crazy. I said as much and and he said no, Harlie is rare. 

So, just how rare is she? Factor in her crazy heart, add her lung disease which required a lobectomy at 10 months old (even more rare) and her Goldenhar Syndrome and that she has a trach and what do you get? 

Anyway, after that was done, we waited a good long while before they let us go back to see her. 

She was "sleeping" pretty well, and it was almost 7pm and we needed to eat. So, we ran and got dinner. Then we went back to hang with her. 

We went to a restaurant down the street that we've been to before, years ago. On the way, we passed the restaurant that we went to when we celebrated Harlie's decannulation (getting the trach out) in 2015. Feels like a lifetime ago. Feels like we were different people then. 

While we were enjoying glasses of wine and good food, it struck me how crazy our life is. I know that most parents who have kids in the hospital probably don't leave as much, or go to nicer restaurants to eat. But, we need moments when we can pretend that we are normal people. Even if it is for just an hour. 

On the way back the nurse called me to let me know she was waking up. So, we hurried back. She has to lay completely flat for a certain number of hours (I think six?). 

Then they get some xrays, and they have to remove one of the pressure dressings (she has two). That meant that they would have to get her up at almost midnight to get her over to Radiology. She was pretty miserable. 

But, she is so good and cooperative and she just powered through. I explained everything as we went and she listened and did a great job. As soon as we were all done messing with her, she put her sleep mask on and went to sleep. 

OMG. Just look at her sats!! This is on ROOM AIR!!! I have NEVER seen her sats this high.

94?!?! On room air!!! 

I really never even thought this was a possiblity. Didn't even allow myself to dream of it. She's never been able to tolerate standing in the shower, so she's always taken baths. It is a possiblity that she could handle showers now. Not to mention we won't have to turn on the oxygen concentrator every night. So crazy!!!

Wow. I'm just absolutely blown away by this turn of events. 

I "slept" in the chair next to her and Tom went to the hotel to get some sleep. He showed up bright and early with coffee and a smile. 

We got her all cleaned up, took out her two IVs, removed the 2nd pressure dressing on her jugular and talked to Dr. Kanter again about the plan moving forward. So, all in all a great stay! 

We have a 10:30 appointment with the advanced lung team/pulmonary doctor. Dr. Kanter said the adult congenital cardiologist was going to try and pop in to meet us while we are there. 

Since we have some time to kill, we came out to the garden. 

I'm going to wrap this one up here, so I can focus on this next appointment in a few minutes. 

Thank you so much for following along and letting me share all my feelings - the good, the bad and the ugly. I appreciate it more than you know! 

Much love, 

Christy xo

Change in plans...

Warning, this one isn't going to be fun to read. I need to write everything down so I can process it. But, it is going to be medically heavy.

We had to get through the three day weekend, which wasn't easy. I thought for sure I would hear something from her doctor on Tuesday since they probably received the results of her CT scan. Then at 6:30pm he called. He asked if Tom was with me and I said yes. He asked if he was on speaker and I said yes. Clearly this was going to be a lengthy call and he wouldn't want to do it twice. Totally understandable. 

He said they had conference and went over her CT scan, her most recent Echo (done in April), and her heart caths (many over the years, but her most recent one was in 2022, I believe). Here's where we are:

1. Pacemaker Revision: the surgeons feel that because of her heart anatomy (her heart is not normal - it is in a mirror image and has all kinds of craziness to it) they feel that the only way they can access her ventricle is by doing a full sternotomy (where they cut through the sternum to gain access to the heart). So, since they must do a full sternotomy, they might as well replace the atrial lead as well. So, that's the plan as far as her pacemaker goes. 

2. She has leaky valves in/around her heart. I've heard "tricuspid regurgitation" for years. Seemed like it was mild and not a concern. However, she now has some regurgitation with her aortic valve, or it is worse than it was. I don't remember ever discussing this issue. So, it is probably new. 

3. Her Fontan is too small. I really don't know how to explain this to people who have no idea what a Fontan is. There is a post I wrote that gave a basic description of her heart here. But, I really don't expect anyone to read that. So, the simplest way, I think is: 

In a normal heart the right ventricle pumps blood to the lungs to get oxygen. The left ventricle pumps the oxygenated blood to the body. But, she didn't have two ventricles. She had a VSD (ventricular septal defect) that is essentially a hole between the ventricles. Hers was so large, she was really a single ventricle. Basically, she only has one pump that goes to her body. The Fontan is a surgical procedure that re-routes the blood flow so that the blood coming back from the body (which needs oxygen) goes straight into the lungs first, to get oxygen, then goes to the heart to be pumped back to the body. 

Because of this new circulation, blood flow to the lungs depends on pressure and smooth circulation. It is definitely less efficient than normal circulation. Over time, the pressure (which can often be elevated) puts a strain on other organs especially the liver. That is called Fontan Associate Liver Disease (FALD). Unfortunately, Harlie's pressures were elevated for years. And we know she has FALD, and that her liver is congested and appears fibrotic on scans. 

Anyway, her Fontan being too small (think highway reduced to a single lane road) is a big deal and they cannot ignore it. So, since they have to do a full sternotomy, do they need to address her too small Fontan while they are in there? Well, a Fontan revision is a major surgery. So, they want her to have a heart cath first. They want to see if they can help her Fontan by putting in a stent to open it up and they are thinking that can be done during a cath. Her doc said she got a stent several years ago, but I don't remember the specifics. Well, I just searched my blog and wouldn't you know - I found it. Here is the post about it!  Now I remember! Haha! They also want to do an esophageal echo where they do the echo from inside her body versus on top of her chest (which does not give them great images/info). 

Also, if you read the post from December 2017, spoiler alert - she still has exercised induced intolerance. Just today, I asked her if she would like to go for a walk with me (well, she rides in her chair and I walk next to her). She said sure, but she had her pjs on. So I said you need to get dressed. She was like, ugh. So, I asked her if she wanted me to go upstairs to get her clothes and bring them downstairs for her and she said yes. That is not her personality. She is very independent and likes to do things for herself. But, physical exertion - that's a different story. She just can't. 

Anyway, they are trying to move some patients around to get in her next week. Then, based on how the cath goes and if the stent works, they will make a surgical plan. As of right now, they are thinking she would have surgery in mid-June.

Right now, we are hoping that the stent works. If it doesn't, then we might be looking at a Fontan revision and that sounds really ugly and scary to us. 

I just received a call that her heart cath is scheduled for Tuesday, June 2nd. 

Okay, so here's how I'm feeling. Terrified. I never want to speak for Tom, but he's probably terrified, too. Look, we knew all along that her heart was going to become a bigger issue. But, that does not help. Not to mention that this essentially came out of nowhere. We were doing just fine and now I feel like the wheels are coming off! We are not prepared for the what ifs, nor will we ever be.  No prior knowledge makes the future hurt less.

After we spoke to the doctor, Tom called his mom. And I realized that I can't call my mom. There is no one who loves your children like your parents. My mom loved Harlie so much. She was so proud of her. She was so impressed by her. I'm so grateful Harlie has her Grandma and her Grandma loves her like my mom did. But I am sad that she doesn't have her Nana anymore. I'm sad I don't have my mom to gasp and cry and have all kinds of reactions that - at the time - I called dramatic. 

Okay, well that's it for this one. I'm happy I was able to get her outside for a few minutes. Plus, we got to see a cute little family of geese. 

As always, thank you for reading and caring. 

Much love, 

Christy xo

Pacemaker Update

There have been some new developments regarding Harlie's pacemaker. A few days after our appointment in Fredericksburg, I read the clinical note from our appointment. A few key things stood out to me. 

1. She wrote that while her goal is for Harlie to be active and do all the things she would like to do, she could not say that it is safe for her to travel out of state for camp. 

2. In the plan bullet points it said if we do not urgently go for pacemaker revision, then I would like her to be seen again on June 4. 

3. Any fainting should be treated as an emergency with 911 and CPR.

It said other stuff, too, but those three were the ones that made me think. 

Then on Friday evening (like around 6pm) I received a text with a secure message from her doctor that she wanted to call me later that evening. I was like, huh? It is Friday night. That is highly unusual. So, I replied, of course, call me whenever it is convenient for you. Well, she didn't call. Later I looked at Harlie's phone and it appeared that my reply never went through. So, that didn't sit well with me. Obviously, I had to go through the whole weekend wondering. Also, now I'm afraid to leave Harlie alone at all. What if she faints and I'm not there to call 911 and do CPR?!?!?

On Monday morning I was able to speak to her doctor. She was so nice and asked me how Murphy's graduation was and how I was doing (since I cried in her office). I told her that I have a lot of big feelings and they are leaking out sometimes. She was very compassionate. 

Then she told me that the team got together and discussed Harlie's case and decided that her pacemaker needs to be replaced soon. She said that they just needed to get in touch with Harlie's surgeon in Boston to ask him about manipulating her jaw so soon after her jaw surgery. She was thinking she would need to be intubated (with a breathing tube, the way most people are put under for surgery). So, I said, that won't be necessary, since she has a trach, you won't need to manipulate her jaw. She was like, oh, duh! And she apologized. I told her no worries - that's what I'm here for! Haha! I told her I am like her General Contractor. Then she said, no, you are her air traffic controller. I only know where my plane is - you know where all the planes are. Nice! I like it!

Then she said that they are working on a date and would call me back. The next day her other doctor called me. Her surgery is scheduled for Friday, May 29th. So, no camp for her. That is also two major surgeries in just over three weeks! Her wrist is still black and blue from her arterial line. And I know they are going to have to give her another. Anyway, he went over that they basically have two options. I'm going to try and explain it - really just for my records. It really comes in handy later if I write all this stuff down while I can remember it.  

So, if I understand this correctly, there are two leads, each lead has two nodes (shaped like a Y). I think there are two atrial nodes and two ventricular nodes. The ventricular nodes/lead was placed on her heart when she was just four days old. They anticipated her needing them later. 

As she got older, I think/guess her heart developed other issues that required them to place the arial lead/nodes. Or, maybe they didn't have room to place all the nodes on her 4 day old very tiny little heart. The atrial nodes were placed in March of 2012, when they placed the pacemaker generator and officially started the pacemaker. 

So, I believe (if I remember all this correctly) that her ventricular nodes are 19 years old and the atrial nodes are 14 years old. The ventricular nodes are the ones that are failing, thus they MUST be replaced. However, the atrial nodes are still working, thus replacing them becomes more of a choice. If they just replace the ventricular nodes, I believe they can do a half sternotomy. If they replace all the nodes, they have to do a full sternotomy. Clearly, one is more involved than the other. So, do we replace just the ventricular ones - or both? Apparently, replacing both doesn't automatically guarantee that they will last for the next 10 years. They have replaced nodes/leads and they have failed after just one year. So, we don't automatically get that comfort of long lasting nodes/leads. It sounds like either way, whether they replace the atrial ones or not, they could still fail at some point. 

I asked him if her heart is adhered to her chest wall by scar tissue, does that factor in to any of their considerations. He said he could not find the note that actually confirms that is the case. He asked me when we were told that. I told him I couldn't remember, but I would check to see if I wrote it down somewhere. haha. So, I searched my blog and found it - March 12, 2012.

Anyway, the next morning he called me to let me know that he ordered a CT scan and that we could do it at VCU to save us a trip to DC. While I had him I asked him if he would mind calling Tom and explaining what he explained to me the day before. When I explained it to Tom he had questions, and I did not know the answers. He said he didn't mind. So, later that day he called Tom and went over everything. By then, he said he did find a note in her chart about the adhesion to her chest wall. Great, so I'm not crazy. I believe he told Tom that if that is the case, it complicates matters a bit. Maybe things can change? I don't know. So, we went yesterday (Thursday) and had the CT scan done. Her doctor told Tom that they hope to have the results soon so they can make a plan. They are supposed to call us early next week. 

As of now, she has a pre-op appointment at 9am on Thursday in DC and then surgery Friday am. We are working on getting a room at the Ronald McDonald House there, but I don't think they can confirm anything until next week. 

What I do know is that she will not return to school this year. It is just not worth the risk - of her getting sick or her just wearing herself down more than absolutely necessary. On Wednesday, she didn't leave her room. When she has the energy, she will come downstairs. She clearly didn't have the energy. Then yesterday, I made her leave because she had the CT scan. We'll see what she does today. So far, she is still in her room. This is two major surgeries in just over three weeks - and that is a lot for her. 

Regarding camp - clearly she cannot go. When I found out, I emailed them and asked if they would please consider letting her return next year for her last year and I couldn't believe it, but they said yes! We didn't want to tell Harlie anything until we had more information (like a date and an answer to the camp question). So, when we had those, we told her. She took it well. She asked where and we said DC. She said that they called her cell phone that afternoon but she didn't answer it. She said, "It wasn't any of my business, so I didn't answer." We had a little chuckle because it actually is her business. Anyway, I am pretty sure she doesn't understand that she will have some version of a sternotomy. I'm guessing that is a coping mechanism - keeping herself in the dark when she can. 

Back to yesterday (Thursday), I've been trying to get her scheduled for a haircut, but it really hasn't been a high priority and we just haven't had time lately. I did finally schedule it for June something - but now with her surgery next Friday, I really wanted it cut before. Her hair can be a challenge in the hospital. There's just so much of it! After her TMJ surgery, it was full of blood. I have washed her hair so many times since, but I feel like I can still smell it! Anyway, I thought a cut would make life easier post-op. So, on a whim I asked my person and she happened to have a cancellation at 4:30 yesterday. Wow - that is perfect! Her CT scan was at 3pm, so as soon as that was over I just stopped at the salon on our way home. Wow. Things rarely work like that! 

So funny, as I was walking up I saw Melissa (she cut my hair for YEARS before she made a career switch). Then I walked in and Donna was there getting Alex's hair cut! How crazy is that? And then Patti was there getting her hair done. How crazy is that?! It was such a fun way to end the day - seeing some of my favorite people! 

Well, last thing - we got Murphy all moved in. Well, there are still a few things here he might still get. But, for the most part, he's settled into his apartment. I have been adjusting to the little things - not leaving a light on for him (since he would get home so late when he closed), not knowing that he got home safely after work, and of course, not seeing him everyday. I know it will get easier, but I still miss him anyway. 

Also, I feel the need to just say to all my loved ones, that I'm pretty focused on us surviving right now. Things are little overwhelming at the moment. So, I'm not asking how you are or what's going on in your lives and I'm sorry about that. I care - I just can't show it like I normally would. One day, life will calm down again and I hope to get back to being a regular person. For now, I do not feel like a regular person. My worries are big right now and they take up so much space. Thank you for understanding. 

Much love,

Christy xo

Home and life moves on...

Hi. Well, we made it home on Saturday, May 9th (my Mom's birthday). It was a long day. We took an Uber to the airport. When we landed at Reagan, we had to take the shuttle to the rental car place. Then we had to wait there for a while. Then we finally got the car and drove home. I think we got home around 7:30pm. I think it, I say it - every time - but I cannot imagine how hard all that travel is on her after all she's been through. 

The next day was Mother's Day. It was a beautiful day. But, we were just too tired to enjoy it. We did sit outside for a while, which was nice. Harlie didn't come downstairs at all. So, as far as doing something "special" well, that was just out of the question. We needed to catch our breath. To be honest, it was a difficult day. Not only did we just get through a grueling week, with another grueling week of recovery ahead, it was my first Mother's Day without my Mom. It was weird. With all my feelings, I just didn't have it in me to do much wishing other moms a Happy Mother's Day. I stayed off my phone and just rested as much as I could. We had dinner with the boys, Tom made crab cakes, which I requested. The boys gave me nice cards and wonderful gifts. They are so thoughtful and sweet. 

Monday was Murphy's 22nd birthday. He didn't have to work at all, which was nice because that meant I got to spend almost the entire day with him. He asked me to help him work on going through stuff in his room and packing. I also wanted to get him new bedding, so we went to Target and he picked out all new stuff for his bed. He tells me he is going to make his bed every day when he gets into his apartment. Haha! We'll see. We gave him stuff for his apartment, like a set of pots and pans and a cast iron pan that he wanted. He bought a kitchen island off Facebook marketplace and Tom fixed it all up for him and painted it. He's in pretty good shape, really.

Harlie's Electrophysiologist (EP/pacemaker doc) moved us to 9am on Tuesday in Fredricksburg so we could get out of there in time to go to Murphy's graduation. 

As far as how it went... it went as most EP appointments go - they essentially say the same thing - the leads are tricky, but they still work. She's stable for now. Honestly, they do an awful lot of fiddling around with her device for me to believe that she's "stable". From my perspective, it seems like they are putting in a lot of effort to turn around and say she's stable. The EP put a Holter Monitor on her for the next 24-48 hours so they can see if they can get more data. She said that she really can't tell me if she'll need a replacement in the next six months or in the next two years. They just have to watch her closely. It's kind of frustrating. No, I don't want to rush into any surgery - especially right now. But, at the same time, I don't want us to end up forced into a surgery with no time to prepare. Or worse - have a catastrophic failure that ends horribly. Or, everything will be fine for the next two years. Seriously? How are parents supposed to live like this?!?!?

I told her that Harlie has camp in Indiana next month. Are we risking too much by sending her? I don't want to live in fear and have her miss out on valuable experiences (this is the last year she can go to camp since she will be 20 in September). But, I also don't want to be negligent and dangerous. We have an anniversary trip to Spain planned for the end of August/beginning of September. Should we not go? What if we go and something happens? She said she wants us seen by EP again on June 4th (her EP doc comes to Richmond on the first Thursday of every month). We'll have that conversation then. Hopefully they will have the results of the Holter monitor by then. As far as our anniversary trip, she said don't cancel anything yet. 

I just hate living like this. When I started to tell her doctor about camp and our trip, I started to cry. I HATE it when I do that. Amazingly, there's only been a handful of appointments when I've cried. On one hand, I feel weak/out of control when I do that. But if you think about how many more appointments when I HAVEN'T cried - I'm like, hey, look at you! Haha! 

The bottom line is that life is kicking our asses right now. Normally I can keep myself somewhat contained. But, I just don't have the energy required to keep all my feelings contained, so my feelings are spilling out all over the place. This makes me want to go home and not leave and not see anyone. 

Anyway, we left there and headed back to Richmond. It was a quick turnaround since Murphy had to be down at the Siegel Center by 1:15pm. Caylee came over to hang with Harlie while we were gone. Of course, Harlie wasn't feeling up to going. She missed Murphy's high school graduation because she was in the hospital. Now she missed this one. Ugh. 

There were about 800 students participating in the graduation ceremony. I assumed the students were in alphabetical order, so when the students first entered and filed into their seats, I wasn't really looking for him. I just figured he would be in the middle of pack. But, something made me look up at the jumbo tron thing and as soon as I did, there he was! It was like intuition. He was in the second row, and we watched him walk in. It was like he could feel us because he looked right up at us and waved. That is so crazy because the place was packed and we had no idea what the set up was or where we would be. I thought there was no way he was going to see us. Crazy! It's like he could feel our love, haha!

I mean, just look at how cute he is!! 😍 Haha! 

I forgot to mention that on Monday, I started to feel sick - like a cold sick. When we sat down, I realized I forgot to grab some tissues. So, Cooper went to the restroom and grabbed me a handful of toilet paper. Classy. Anyway, after seeing Murphy's cute face, I just started to cry. Ugh - there goes my feelings spilling out all over the place again. I mean, I wanted to excuse myself and go sob somewhere private. But, clearly that wasn't an option. I had to get myself together. 

The President of the community college spoke and I really liked what she said. She said she gets asked about the type of student that attends the community college. She said that they are extraordinary. To prove her point, she asked the graduates to stand if they are the first to earn a degree in their family. Then she asked the graduates to stand if they are a parent or the primary caregiver. Then she asked the graduates to stand if they had a full-time job while they were in school. Then she asked the graduates to stand if they were getting their Associates Degree before they graduated high school (there is a program where you can earn your associates in high school). By then it looked like every graduate was standing. It was pretty cool. She also said that the youngest graduate is 17 and the oldest is 72. Pretty awesome. 

Ahh, you gotta love that sibling support. Cooper was just "resting his eyes". 

He chose to go eat at Stella's to celebrate (that is the restaurant where he works). I just love going there because I get to hear so many good things about Murphy. So many people come up to us and tell us how much they love him. It fills my heart and I really needed it right then. 

A candle for his birthday...

Just because I can... here is an old post where I shared some good, young pics of Murphy on his 7th birthday. 

Later that night, I took a down turn and really started to feel bad. I had a terrible night and woke up feeling even worse. This has happened before after a hospitalization. I just get so run down. It is my body's way of telling me to chill out and recover. As if I have any control over that. Trust me, I want to chill out. I love to chill out. I wish I could chill out.

Speaking of not being able to chill out... I received a reminder for her next bronch (a follow up from her last bronch in February). It is scheduled for May 27. I just can't do it. I'm going to have to reschedule. But for when? Camp is June 14. I don't know how or where I'm going to fit it in (maybe after camp if the doc thinks it can wait that long). I just can't do it so soon. She has five doctor's appointments on her calendar before June 12th. That is ridiculous. 

Today is Wednesday and I convinced Harlie to come sit outside with me. 

I don't know when she will return to school. Definitely not this week. 

The next hurdle we have is to help Murphy move into his apartment on Friday. So, we have two more nights of him sleeping in his bed in our house. I know, I know, this is great. It is! I have full confidence in his ability to navigate life. I am so proud of him. But, oh, I am going to miss him so much! Feelings aren't either/or. You can have lots of feelings at the same time - like I am both happy and sad about him moving out. Like I said, we are getting our asses kicked right now. I wish so many life changing things didn't happen in such a short time, but sometimes that is just the way it is. 

Well, I didn't get this finished and out on Wednesday. So, now it is Thursday. Harlie has not come downstairs today. But, I have been able to remove the dressing and not put a new one on. I'm just leaving it exposed to the air now. Tomorrow we will remove the sutures. Hopefully that goes well. I also removed her Holter Monitor and put that in the mailbox to be returned. 

That's it for now. Thanks for reading!

Much love,

Christy xo