Spring 2026 Update

Hi. Just going to jump in here and try not to think too much about it. 

In my last post I listed some upcoming appointments. 

Here are some updates:

Electrophysiologist (Pacemaker) - She's seen him twice since my last post. There is a device near her bed that sends her pacemaker transmissions to Children's National every few months. They review it and let me know that all is okay (usually). In February, I learned that her heart had two events. One she's had before and is on meds for already (NSVT). The other one is new to her (atrial flutter, which lasted about three hours). So, he increased her meds and said we just need to watch her. He asked if she was sick during the time period of her events - and she was! He said we just have to really watch her when she gets sick because it really stresses out her heart. Awesome. 

Hepatologist (liver doctor) - they ordered a CT scan with contrast to try to get a better look at her liver. Normally, they would do an MRI, but they can't with her pacemaker. Basically, they were looking for potential cancer, since she is at an increased risk with the extra pressure/work on her liver due to her heart/lungs. So far, so good. 

She had her wisdom teeth extracted and I am shocked that all went well. No issues. 

Opthalmologist - all stable there. Also, sadly, I have given up on the whole occuloplastic surgery that I was trying to coordinate this time last year. That was a mountain I just couldn't move. Turns out they wouldn't consider trying to do both surgeries in the same week even, which would mean another trip to Boston JUST for her eye. I just can't even think about it anymore. 

Bronchoscopy - she had a bronch in February and it was really just a check up. I don't think she's had a bronch since the whole airway reconstruction in 2018. But, my memory is a little fuzzy. Anyway, her local ENT said it has been a long time and we should just check it out. I almost canceled it. It is hard to work in "well checks" when we are already doing so many appointments. But, for some odd reason, I didn't. Anyway, can you believe that she had so many granulomas blocking her airway!?!?! Wow! I really don't know how she was handling her speaking valve at all. Her ENT showed me pictures and it was crazy. Thank God she suggested the bronch! Harlie is definitely tolerating her speaking valve so much better and I really feel like her voice is so much better! She will have a follow up bronch at the end of May. Unless I reschedule it - because I'm writing this post on May 5th while she is in the OR...

In other news:

My Mom went on Hospice in August. We moved her into a house that has a caregiver that lives there. Mom had her own bedroom. At first it was hard on her (it was hard on all of us, really). But, she finally got to know the people there and they were good to her. She had dementia. My Mom deserves her own post, but I just don't have it in me to write about her yet. She declined through the winter. By February, on my way to visit her I was always afraid she wouldn't remember who I was. The last time I visited her when she could speak, she told me that I looked just like her daughter. She also said I was pretty. It was so hard to drive to see her. It was harder to leave. She passed away on March 1, 2026. 

Even though we knew it was coming, I was so surprised at how hard it was. I thought since I know a thing or two about grief, I would handle it better. Not better - maybe I thought I would be less sad. But, it doesn't work that way. Honestly, I'm still sad. Which is probably why I don't want to write about her yet. 

On March 21, Harlie went to the All-Star Prom (prom for exceptional education students in the county). I didn't commit to her going in time to secure transportation - so I dropped her off and picked her up. Tom was out of town that weekend. No matter what, it is always hard to drop her off somewhere. Is she polite to other kids? Do other kids talk to her? Does she talk to other kids? Will she have fun? Will something embarrassing happen to her? She said she had fun.

April 9th - We announced that we were closing down operations for We Heart Harlie & Friends. This was such an incredibly difficult decision. This is also a subject that I feel deserves it's own post. This also makes me very sad and I'm just not ready to write about it, either. 

April 11 - Since she went to the All-Start Prom, I was kinda hoping she wouldn't want to go to her school prom. But, she did. Since she had just worn that other dress (that she's worn before), I ordered a bunch of dresses and hoped one would fit. Thankfully, one did. Well, it was too long, but I was able to just cut off some material at the bottom to make it work. A seamstress, I am not. 

The prom was at a hotel. Cooper and his girlfriend and friends went out to eat before. So Tom and I drove her to the hotel and dropped her off. Dropping her off at a hotel was way harder than dropping her off at the high school for the All-Star Prom. But, her teacher sent me photos and videos of her dancing and that made me feel better. Cooper drove her home and they all had fun, I'm told. I don't know what I'm going to do when Cooper graduates and Harlie won't have him around anymore. I try not to think about it, because it makes me sad and scared. But sometimes I can't help it. 

Cooper picked out his suit and purchased it himself. I don't have a picture of him and his girlfriend, but he selected this suit because it matched her dress. 

April 23 - Murphy and a friend of his drove to Pittsburgh for one night to attend the first night of the NFL draft. Those crazy kids. It is so funny to remember that just a few years ago, Murphy didn't know or care anything about football. Today he is a Steelers super fan and a football expert. Haha! Thank you to our dear friends Mike and Laura for putting them up for the night!

Murphy came back the next day (Friday) so he could attend Cooper's school play - Shakespeare's Twelfth Night. Cooper played Sebastian. He was so great, as was all of the students! It is truly amazing what these kids can do. We have already been super impressed with their ability to memorize and perform all their lines - but Shakespeare? That's on another level. Anyway, it was great - we all loved it. I went two nights. I just love getting to watch Cooper on stage. 

Cooper is in the center, to the right of the girl in the blue dress.

Cooper and his girlfriend (Riley). She was Antonio in the play. 

Mary Ann (aka Grandma) came down for the weekend so she could see his play. 

April 26 - We went to see the Richmond Flying Squirrels for Tom's birthday celebration (his birthday was on April 28th).

Our friend Dave made this happen. It is hilarious because Tom turned 53. Haha!

May 1 - We went to Banana Ball. It was crazy. It was our first time experiencing the chaos that is Banana Ball. 

It was the Firefighters vs. the Clowns. There were "characters" all over the place. 

I would say that it is more like a show with a side of some baseball-like game. It was fun and different.

What's ahead:

May 11 - Murphy's 22nd birthday. I really hope to be home from Boston before his birthday. 

May 12 - Murphy graduates from Reynolds Community College. Now I REALLY hope we are home for his graduation. We are so incredibly proud of him. He worked full-time while going to school and paid for all of his classes along the way, all on his own. 

May 15 - Murphy is moving out and moving in with a friend of his. Thankfully, he won't be too far away (20-25 minutes or so). I am unable to write about this right now as well. Yes, I am so proud of him. Yes, I am so happy for him and excited for him. Yes, I know he is ready. Yes, I know this is the way it is supposed to go. Trust me - this is not the way it will go with Harlie - so I know to be so grateful for the life experiences that are good, normal, and all that jazz. But, I am going to miss him being in our home and sharing our address more than I can say. This year has already been pretty hard as far as changes go (there have been many) and I am just going to need to feel what I feel. 

This year should be the year that Harlie graduates from high school. But, it isn't. This definitely needs it's own post. There are so many feelings that go along with this. Just know that I'm feeling pretty raw right about now. Some days I find myself so angry - at everything. Then I realize that what I'm feeling is just pain. Pain all over. Every interaction I have just causes me pain. Even when I'm happy for someone else, it just reminds of me of what we've lost. 

Anyway, that's it for now. I'll start working on the hospital posts. 

As always, thank you for reading, thank you for caring, thank you for the love!

Christy xo

Medical Updates

Hi! It has been a while since I've given a medical update. So, here goes...

Temporomandibular Joint (TMJ) Update

Here's a recap of her TMJ saga:

April 2021 - She had her first custom prosthetic TMJs placed on both the left and the right. This was one of her most brutal surgeries/recoveries. This is the hospitalization when her tablet was stolen from her bed while she slept. And this is the one when she couldn't stop bleeding for weeks. It was a horrible time. Anyway...

May 2021 - a growth appeared at her incision sites on both sides. I took her to several doctors to try and find out what it was.  

August 2021 - I finally got her into the OR with her ENT in DC. He said it was an abscess/infection. They gave her a PICC line and a two-week course of IV antibiotics.

September 2021 - the abscess returned, so infectious disease put her on Doxycycline. Over the next year, we attempted to take her off Doxy several times, each time the infection returned, so she went back on Doxy. 

November 2022 - the infection returned on the right side only, despite being on Doxy. After consultation with several of her doctors, the decision was made to return to Boston to remove the TMJ.

April 2023 - the right TMJ was removed since that is the side where the infection re-appeared. The surgeon said the right side had a track and we never would've beat it with antibiotics. He put a medicated temporary spacer in it's place. While she recovered from this surgery, they made a new custom prosthetic TMJ for her. 

October 2023 - the new custom right TMJ was placed, and the left TMJ was repaired since he found it to be dislocated. This was very unfortunate, because it meant that he had to go into the left side, which opens up the possibility of post operative infections.

December 2023 - We were finally able to stop the Doxy! Yay! This was great, because by November, it became difficult to get her Doxy at all. For some reason, there was a low supply, and I would have to go to several different locations to get what she needed. So, when we were finally able to STOP giving her Doxy, we were THRILLED. It was a little scary at first. But, her surgeon said he felt really good about how the surgery went and he didn't have to go into her mouth this time, so the whole site was cleaner, in general. 

January 2024 - The infection reappeared on the left side (not the right). The left side is the one that has the original TMJ and was dislocated. When I was ready, I contacted our infectious disease doc and she put her back on Doxy. 

December 2024 - On Christmas morning, I discovered that the infection reappeared - even though she has been on Doxy consistently since January 2024. 

January 2025 - I contacted her infectious disease doctor. She put her on Linezolid for 12 days and after that course, we went back on Doxy. The infection looks unchanged since December. So, I had to reach out to her surgeon at Boston Children's Hospital. I explained to both doctors that we are scheduled to go to Hawaii for her Make-A-Wish trip in March. There is no way we have time for her to have surgery before then. So, can it wait till after the trip? They both felt that it would be okay to wait since she will remain on Doxy. 

Trying to find a "convenient" time for this surgery was a challenge. Tom will miss a week of work in March. Then he starts a big job as soon as he gets back. This meant he didn't want to be away in April. We Heart Harlie & Friends has a major fundraiser April 26, so I didn't want to be away during that. She also has summer camp in June. So, she needs to have the surgery before and have enough time to recover from that before camp. I hate for her to miss school - especially if it is the end of the year. But, we really had no choice. 

May 13, 2025 - She is scheduled to have her original custom prosthetic TMJ removed and he will place a medicated temporary spacer. They will then make a new TMJ for her and we will have to return to Boston (hopefully before it gets cold up there) to have the new one put in. We will have to talk about what he can do to try and prevent the right side from becoming dislocated during this process. I fear that we will be doing this forever if we can't prevent a new infection from brewing on either side. 

I have to admit that I am NOT looking forward to returning to Boston again. I just don't know how many more trips and surgeries we can handle. This is not something I would ever choose for her at this point. It is out of my control. We cannot leave an infection in her jaw. But, I am so DONE. I say that, but I know I can't be done. I have to make her go again - and then again in the summer/fall. 

Harlie's Left Eye

One of Harlie's original birth defects was that her left eyelid didn't form correctly. When she was born, she actually couldn't close her left eye on her own. After a few months, she was able to gain some closure. But, it has never fully closed. This leaves her eye vulnerable to dust and particles, etc. When she was in the hospital all those months in 2017 and 2018 (two medical induced comas - one lasting 7 days the other lasting 22 days) they had a hard time figuring out a way to protect that left eye. They tried taping her eyes shut, putting masks over her eyes, sometimes both at the same time. But, her corneas still ended up with scarring. She sees an ophthalmologist yearly. 

Well, this past fall her ophthalmologist said that her scarring has gotten worse and that she was surprised she could see as well as she could out of that left eye. She explained to Harlie that she must put eye gel in her eye before she falls asleep every single night. Surprisingly, she listened to her doctor and has been really good about it - even though she hates it. However, her doctor said she thinks it is time to talk to a surgeon to see if something can be done to her eyelid to help with closure, which would hopefully stop/prevent more damage to her cornea. 

So, when we scheduled her TMJ surgery in Boston, it occurred to me that it might be good to get a consultation from a surgeon there, who likely sees more kids with these kinds of defects. So, I asked her plastic surgeon about it. They told me that she would need to see an oculoplastic surgeon. So, I called that office and asked if we could schedule a consultation. Not surprisingly, this has proven to be a difficult ask. 

I was thinking they could come and take a look at her while we are there for pre-op/surgery the week of May 12th. Then, IF they felt that they could help her situation, they could schedule surgery at the same time she is getting her "final" TMJ placed in the summer/fall of 2025. 

Of course they asked that her records and notes from her eye doc here be sent there for review. The office in Boston told me that they are scheduling appointments for November 2025 at this point. But, he is going to talk to the team since we are traveling and ultimately, I'm trying to minimize the number of surgeries she has to have. After a few days, I received another phone call and he told me that there are three oculoplastic surgeons. But, only one of them can (?) operate on patients who are 18 (the other two are pediatric only) and that it looks like she (or all of them?) is away at a conference that week. He asked if we could come another time. I told him that it is extremely difficult and costly for us to go there and if I can't combine visits, I just can't make it work. I'm sorry, I just can't. That would be a THIRD trip to Boston in one year (when I want to go zero times) and I am only human and I'm sorry that is just TOO much! 

I cannot travel with Harlie alone - I need Tom. And Tom is missing work in March, then again in May, then again in June (to take her to camp) then again to take/pick up Cooper from camp in Maine (he was selected to be a counselor in training at camp this summer!), then again for her 2nd stage surgery. Also, keep in mind that I don't even know that they can help her and I don't even know if insurance would approve it, etc. All of that is really an impossible schedule. 

He said he understood and he actually sounded pretty sympathetic. So, he said he was going to talk to the team and get back to me. Honestly, I don't know what he can do. Sounds like a pipe dream that isn't going to happen. Well, you can't say I didn't try. 

Guardianship

So, now that Harlie is 18 and she isn't a typical 18 year old, I have to think about a guardianship. This has been difficult for me because most of the time when you hear about a guardianship, it refers to an "incapacitated" adult. Even though Harlie is delayed and has a heck of a complicated medical life, she is not incapacitated. However, according to her recent school tests, evaluations and IEP, she is very delayed (she's basically on a 2nd grade level and she is in the 11th grade). I'm thinking that an adult who is operating on a 2nd grade level could benefit from some protection financially and she definitely needs help navigating her medical complexities and decisions. 

But, as usual, nothing is simple. I've now been working on this for months and I feel like I've made no progress. Medically, we don't have a diagnosis that explains that she is delayed - or why she is delayed. She has no diagnosis of a learning disability or anything neurological. All of her IEP and school stuff talks about what a hard worker she is, how much she wants to learn, etc. So, we need to get a psychological evaluation to show her cognitive ability/IQ and adaptive skills. I'll spare you the agonizing steps I've taken to come to the learning that most facilities who do this testing in our area are not approved by Medicaid (which is what Harlie has because of her medical qualifications). So, I asked if I could just pay for it privately. The answer is no - because legally, they aren't allowed to bill patients who have Medicaid. So, we can't get what we need? WTH? 

So, that's where we stand. My attorney said that we can start the process with what we have and see what happens.  So, I guess that's what we'll do.  There are times when my life seems unreal and ridiculously overwhelming. I mean, one of these issues would be a lot to deal with. And these are just the ones I'm talking about in this post! 

Cardiology

Harlie had her annual cardiology appointment on Tuesday, February 11. She had an echocardiogram (an ultrasound of her heart). I think that took over an hour, but it felt like forever. 

It was actually interesting because the person doing it was training so I got to hear what they were saying about the echo while it was happening. I gained a new respect for the skill they have to have to get good pictures and videos of a heart - especially when there are challenges like tons of scar tissue and an unusual heart anatomy. However, even though I knew that, after a while I started to get a little concerned that maybe some issue has developed since her last echo and that was causing it to be even more difficult. 

It made me remember when I was pregnant with Harlie and we were sent to Children's National for a fetal echo. We did not have any idea that her heart was wacky at the time and the echo took forever.  In fact, the room is dark when they do it and there is always a hum in a hospital and I actually fell asleep for a few minutes during the exam! Then the tech stood up and said, "huh, the baby isn't letting me see all four chambers of the heart, I'm going to go get the doctor" (or something like that) and then the doctor came in and did the echo for a while then he asked us to go wait in a conference room where there was a box of tissues on the table. 😑 Just in case you don't know, Harlie's heart didn't have four chambers.

Anyway, back to present day, finally they were done her echo and we returned to the regular exam room. When her cardiologist came in a few minutes later, he said, "Her echo looks great." Haha! Ah, the emotional roller coasters I ride when no one else knows I'm riding them. 

Then he said something about since she's 18 now, it is probably a good idea to start thinking about getting a heart cath done to take a look at things. Honestly I don't know how I could work in a heart cath in our schedule right now. It is hard to remember exactly what is said in some of her appointments. Especially after I ride a roller coaster in my brain. But, he said something about her liver and monitoring that and I swear I was like, "Oh yea, I forgot about that issue!" For a little while after the appointment, I reflected on how I could forget such a thing. I mean, it was something that REALLY worried me last year, for quite a while. We've had numerous appointments regarding that issue in the past year. But, honestly, it is survival. I have to compartmentalize and there are only so many tabs I can have open in my brain. Sometimes I just have to say, "that isn't today's problem." 

Anyway, since I had him in front of me, I asked him about my guardianship challenge. I wanted to know what the worst case scenario was if I cannot get it, medically speaking. He said medically, he doesn't think I'm going to have a problem not having a guardianship for her. He said he will write up something for me that explains that there are several factors (just within the cardiology aspect) that contribute to her developmental delay. Like prolonged oxygen deprivation (she has had low oxygen saturations her entire life), cardiac arrests, surgeries, etc. all contribute to brain development issues. 

While it would be kind of nice to get some answers (not that I ever really expect any) as to why she is the way she is or exactly where is she IQ wise or cognitively, he thinks I could put this on the back burner as we have more pressing issues (obviously). I do love when a doctor can stand back and look at the whole picture of Harlie, versus just zeroing in on their specialty. I really love this cardiologist and trust him and I'm grateful to have him in our corner.

So, we'll just see what happens. I've reached out to another contact about the psych eval and maybe one day I'll get it worked out. For now, this is where I'll end this post. I wonder if y'all get as exhausted reading some of my posts as I get writing them. I swear, sometimes I still can't believe this is our life and that we've been living it for over 18 years. 

Thank you for reading and for your continued support! We couldn't survive without it!

Much love,

Christy xo

Here's a pic of our snowy view from the 4th floor of the Children's Pavilion downtown.

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