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