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.
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
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