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

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

Right TMJ Replacement Surgery

Wow. I'm so sorry, I never updated the blog during/after her last surgery. I have worked on some posts, but haven't hit the publish button. 

Well, for now here's the summary:

In March 2023 she had her right TMJ removed (infection) and he put in a temporary spacer. Her recovery was okay. Certainly not as bad as the original placement of both TMJs in April 2021. Which is great, because that was a nightmare. Anyway, if my memory is correct, Tom had a big job starting a day or two after her surgery, so he had to fly home. We were planning on him returning after he got the job going to help us get home. But, that didn't work out. I think it was going to be way more costly for us to wait for him to fly up (another night in the air b&b) and then have all three of us fly back home. So, I said I was just going to have to be a big girl and get her home by myself. 

Well, I'm not going to do that again. That was awful. I just don't have enough hands to push her in her wheelchair and pull her oxygen concentrator and luggage - impossible! I remember getting out of the Uber and having to unload everything (including putting her wheelchair back together) and then I would take the luggage a ways, then leave it, go back for Harlie, then take the luggage further, go back for Harlie, etc. Some nice ladies helped me after they watched me for a sec, so I was grateful for them. But, even after checking the luggage, I had Harlie and the oxygen concentrator. By far, the worst leg of the trip was getting OFF the plane. The ramp that you walk up from the plane to the gate is steep and has bumps from one ramp to another. I couldn't get Harlie up the bump with one hand (and pull the concentrator with the other). I REALLY struggled and finally one of the employees came to help me. He pushed Harlie up and I got the concentrator. Sounds nice, right? No, the worst part is that he looked completely annoyed at having to help me. It was the WORST. 

Anyway, we learned - not going to do that again. 

Okay, back to current events... We flew up early Monday morning. We had to wake up at 3:20am to get us ready and to the airport by 5am. Even with us having TSA, going through security with her takes forever. We got to Boston with plenty of time before her first appointment at 10:30am. So, we took our luggage to the hotel and put it away for the day. Then we went to get breakfast. Then headed to the hospital.

She had several pre-op appointments...

I haven't been able to update you on what's going on with her pacemaker - but back in August it was confirmed that one of her pacemaker leads is going bad. The leads went in during her very first heart surgery at just four days old. So, those leads are 17 years old now! I'll have to save those details for another post. Just know that this is an issue (which will require open chest surgery at some point in the future) and another thing they just wanted to be on top of. 

We went and got dinner and then watched Narnia with Harlie. 

We had to be at the hospital at 7:15am for an 8:45am surgery time. 

I feel like I look so tired in this photo. It is because I am. Haha!

I forgot to mention that back in the summer, her earring in her left ear worked its way out of her tiny little lobe. So weird. No trauma, no big earrings, we have no idea. So, I sent her surgeon a picture of it and he said he could fix while he was in there. 

All that went fine except they were delayed because they were trying to find her an ICU bed to go to post op. They wouldn't give the green light to start until that was confirmed. During the wait, I reminded her that she's going to hurt for a few days again. She said, "I know." Then I told her that in time, she will feel as good as she does right now. She said, "I know." She's so good, y'all. She's so good waiting for surgery. She never complains about being hungry - or about anything at all. It really struck me how good she is (and has been her whole life) while we had to listen to the parent next to us talk on speaker phone (ugh) and her child yelled and screamed that she was hungry and wanted to go home. Now, don't get me wrong - I kinda felt bad for her. No kid wants to go through surgery. But, it just reminded me that we have NEVER had to deal with Harlie like that. Even when she was a baby, or a toddler. It is like she just knew she had to deal with it. 

Anyway, they finally took her back at 10:30am. They said they anticipated a four-hour surgery. We went downstairs and got some food. Tom's messenger bag was breaking, so we went to REI to get him something that would make it through this stay. It was funny, he bought that bag during her first surgery in Boston in 2012. Ahhh, the memories. We had NO idea we would STILL be working on getting her a better airway 11 years later. Anyway, sitting around a waiting room all day has gotten so painful. We've just done it too many times. So, we spent most of the day outside, walking around. We got phone calls every 90 minutes to let us know what was going on. 

Dr. Resnick came to get us around 4pm. He said overall things went well. He replaced the temporary spacer with her new titanium TMJ. He said sometimes when one side doesn't have as much support as the other, it can dislocate the "good" side. Which is what happened. He thought that would be an easy/fairly quick adjustment/fix. But, as everything goes with Harlie - it turned out to be more complicated. He said the dislocation had clearly happened months ago and that soft tissue had grown all around the joint. So, he ended up having to make two incisions on her left side and had to manipulate that side way more than he thought. That means she will definitely have some pain and bruising on that side, too. But, he was able to wash the left TMJ and fix it, so that's good. In total she has four incisions, two on each side. 

He said anytime you're working around/in the mouth - it is very hard, if not impossible to keep things sterile. But, he didn't have to work in her mouth this time. So, hopefully, she will be okay as far as infection goes. We will keep her on her antibiotic for another 4-6 weeks, just to be on the safe side. She's been on this antibiotic since August of 2021, so we will be happy to be done with that medication! 

Anyway, Dr. Resnick said that unfortunately, they still didn't have an ICU bed for her! So, she was hanging in the OR until they could find her one. It is now 6:15pm, and I don't think she has an ICU bed yet. She is supposed to go to the cardiac ICU (CICU) but we checked in with them and they said she hasn't come up yet. Crazy! 

I think when I blog and share stuff with you, I really try to focus on the positive. It is better for me to do that for my own good, too. However, there is always bad stuff. We definitely worry about her. I mean, worry about something going wrong. She's had well over 100 surgeries now and so many of them have been really big ones. She's getting older and her body has been through so much. We can't help but fear something catastrophic happening. Especially when we've had several of those experiences now. 

So, at 4pm we were told that she was just hanging out in the OR, waiting for a bed. Then, I don't know, an hour or so later we were told that she had a bed in CICU and that we should go get our badges, then go up to the CICU waiting area. When we got here (just after 5pm) the CICU said they didn't have her as a patient yet - so she hasn't come up. Now it is 6:30 and she's still not up?!  What the hell? I'm telling you, this is like a roller coaster from hell. We worry, we're told all is okay, now we're worrying again. The worrying about her well being is EXHAUSTING. Add that on to our physical tiredness and I don't know how we live like this! 

Okay, I just went and asked again. She told me that she JUST got up to the CICU and they are getting her settled now. Whew! They will call us when we can go see her. Geez. This life...

I think I'll sign off for today. I'll write again tomorrow. 

Thank you for all the love, thoughts and prayers! We appreciate you all more than you know!

Much love,

Christy xo

Vacation in June and an Update

So long without a post!  Ugh!  Our life is full and busy, which is good I suppose.  But, to be honest, blogging about it hasn't been appealing to me for a long time.  I have been in a crummy place for a while now.  And time.  Time is such an issue.  My office has been taken over by Harlie's schooling and the kids watching Minecraft videos.  What in the hell is up with that?  I just do NOT understand that game.  And between my part-time job (granted, very part-time) and it being summer with the kids home, it's extremely difficult to get in front of the computer.

Anyway, I'll just start talking and see where it takes me...

Vacation, June 14-21

We went on vacation to Lake Anna with my family the week after school let out.  Tom had to work most of the week, so he wasn't there much.  Luckily, since it's only about an hour away, I had nursing for the week.

It was so great to spend time with siblings and my nieces and nephews.  They are such a fun group of kids and we just don't get to see each other as much as I would like.  So, that part was great.  The part that wasn't so great was how little Harlie was around us.

When we got there (it was a big house on the lake, with a really nice boat house) we found this float? tube? not sure exactly what it is intended for, but it was perfect for Harlie.  It had a mesh bottom, so it basically became a kiddie pool in the lake for her.

In that picture she wanted to sit in an additional float, just for fun, I guess.  Anyway, this was a perfect solution to keep her cool on hot days.  Although, to be honest, it's hard to relax around water with Harlie.  It's never far from my mind that everything could change in an instant.  Especially in lake water!  Oh, and someone told me after that week that kids with g-tubes shouldn't be in lake water because of the risk of parasites or something.  Awesome.  Luckily, there were no issues.  But, sheesh!  What's a girl with a trach and g-tube supposed to do for fun?!?!

Anyway, we got there on Saturday and she only spent some of Saturday and Sunday in the float.  After that it got too hot for her.  And some dragon flies came out, and she was done after that.  The girl does not like bugs of any kind.  The rest of the week she spent in the house with her nurse.  In the room where she was sleeping there was a TV with a DVD player.  She thought it was pretty cool to lay in bed and watch movies all day.  While that KILLS me, I try really hard to look at it from her perspective, and if she doesn't get to do that at home, I suppose that's what vacation is all about.

The other thing was that the house was not very close to the water.

Here's the view of the house from the dock.

And here's a view of the house and boat house from the water.

We took a wagon to help get Harlie and her stuff from the house to the dock.  But, with the oxygen concentrator, suction machine, etc., it was labor intensive to get her there and back.  And it was a hot week.  And Harlie just can't handle the heat.  Even when she goes into the water, she can't get her head wet.  So, it doesn't have the same cooling affect like it does for us when we can just jump in.  Anyway, it made me sad that she couldn't be with us, enjoying the same things we were enjoying.  And then I felt guilty for spending so much time away from her.

I know she still had fun.  She is always happy, that's for sure.  And I had a great time, too.  I got plenty of down time, and quality time with my family (minus my husband, unfortunately).  And I even got in all of my training runs!  Which I think is pretty impressive considering it was super hot, not good running terrain and I had my fair share of coconut mojitos.

Harlie got to fish, which was pretty much all she wanted to do.

Harlie's first fish!  And the only bass caught that week!

This girl cracks me up.

Dawn and Harlie

Cooper got in on the action, too!

My Mom and Dad got to ride jet skis...

My Mom (taking time off from chemo) and brother, Bruce.
It was awesome to see her have fun!

My Dad.

My Dad and Tom.

We went tubing.  Which is crazy.  Because I am just too old for that crap.

I am way too afraid of hurting myself.  I'm just not into that.  I thought that since I was on the tube with the FIVE year old, I was safe.  Apparently I was wrong.

I mean, look at this!  Poor Murphy!

Okay, maybe he thought that was fun.

Now, here's where it got real.  My new, and only pair of "nice" sunglasses that I've ever owned (that Tom got me for Mother's Day), flew off my head, never to be seen again!  I have included this picture for your enjoyment only.  Not mine.  Just look at the agony/fear on my face!!!

Yes, they told me not to wear them.  Did I listen?  No.  I repeat, I was on the tube with the FIVE year old, I thought it was going to be a nice, leisurely ride.  Clearly, I thought wrong.  

Apparently, while I may be tough on the inside - I'm a wimp when it comes to water sports.  And now I'm back to wearing cheap Target sunglasses.  Oh, the agony!!!

We sat on the dock and watched a storm come in.  We waited till the last second and then ran for the house.

One night for dinner we went via boat/jet ski to a restaurant on the water.  It was a night when Tom wasn't there and I didn't have a nurse.  The boys went on the fast boat, and Harlie and I went on the pontoon boat.  She loved it.

My niece, Jordan, just because I thought
this was such a good picture of her. 

Bruce and Nancy on the jet ski next to us.

When we docked at the restaurant, I realized we didn't have Harlie's chair.  So, she tried to walk.  But it was far.  So, everyone helped me by carrying something (her suction machine, my bag, her oxygen tank) so I could carry her.  It really wasn't that far.  But, it was to her.  And it makes me so sad to see how little endurance she has.  Any exertion at all is taxing on her and she just can't do it.  One second you feel great, enjoying a boat ride, and the next you get a slap in the face at the gravity of your daughter's heart and lung crappiness.  Sometimes it just sucks more than others.

Our last day was Friday.  Tom had to work and could only come out for half the day.  He brought Rooney with him.  So, he got to spend some time on the lake.

Not too far out in the water there was a table and volley ball net.  So, we played a game of beer pong.  Or two.  The under-aged kids just watched, of course.

Me and my beautiful nieces, Kelly, Maggie and Jordan.

Cooper with Poppy (my Dad).

Tom and I with the boys and Rooney.

Doesn't Murphy look so old in this picture?
Skinny.  But, old.

And Rooney liked it, too.

As did Harlie, of course.

I could so do lake living.

When Tom first got there around lunch time on Friday, he pulled into the driveway and him and Rooney came straight to the dock.  Harlie was inside with Dawn.  I was so excited to see if Rooney could swim that we put him immediately in the water to see what he could do.  He can swim, I just don't think he really enjoyed it that much.  So, I took a very short video on my cell phone and we didn't make him do it again.  Then Tom went up and got Harlie and brought her down, so she could see Rooney.

Hours later, we played and packed up and left.  Harlie and Rooney were in my car and the boys were in Tom's truck.  Harlie wanted to see the pictures on my phone (something she likes to do) so I gave it to her as we were leaving the house.  She came across the video of Rooney swimming and started to cry.  Like a legit, "I'm so sad and disappointed" kind of cry.  I turned to look at her and she said/signed, "Mama, I want to see."  She missed it.  She was in the house when Rooney was in the lake.  And she missed it.  And she knew it.  It's moments like these that kill me.  If her body were stronger - she would have been playing outside with the rest of us and she would have been right there.  With little to no effort.  Instead, just playing outside can be too much for her.  UGH!!!  How did this happen?!  Why?!???  Such futile questions.  It just sucks.  Plain and simple.

There's a bunch of other stuff I want to tell you about, but this is already pretty long, so I'll finish with a "quick" update on Harlie, medically speaking.

I can't remember if I already told you about Harlie's decreasing heart rate.  But, just in case, we realized in May that her heart rate was slower than it used to be.  And hanging out around 60 bpm during the day.  Seemed pretty low to me.  Her pacemaker was set to fire if her heart rate dipped to 50 while she slept, and 60 during the day.  If her heart rate was hanging around 60, that meant it was because her pacemaker was doing the work.

So, on June 25th, we went in to see her cardiologist.  He read her pacemaker and agreed, it was too low.  Basically, due to her heart disease, they expected that her heart would need the pacemaker full-time eventually.  Which, is where she is now.  He said that her heart was working on it's own only 12-14% of the time.  I think since she's now at a place where the pacemaker is taking over, he has more room to control it.  They've always wanted her heart to do what it could on it's own, then be assisted by the pacemaker.  It has been a struggle since her heart initiates a beat from all over her heart, at random paces.  So, the pacemaker couldn't predict what her heart was going to do.  Now, I think he can just set her pacemaker to do what is best, and it's pretty much taken over full function.

After making some adjustments, he said to make her walk up and down the hallway a few times so he could see what happens.  Brandy took her so I could chat with him without Harlie being able to hear us.

I want to know how long we have before things get bad.  But, he can't tell me that, of course.  I asked him what I should be doing and he said there's nothing I can do to change the way her lungs are.  Maybe her left lung will be strong enough to support her one day.  Who knows?  But, eventually, we will be at a place where our only hope/option will be a heart and lung transplant.  If only he could tell me when.  He said he would want us to think long and hard before going down that road.  It's a hard road, with a lot of pain for her, and not good outcomes.

I told him I can usually stay positive, but this latest info is kicking my ass.  I guess her doctors could tell that I had so many hopes and dreams for her.  I really thought she - we - were going to conquer all her challenges and one day, live a normal life.  I suppose from a medical professional perspective (who has seen way more than I ever have) they could see that I was living in la-la land.  The odds are just so against her.  There are too many challenges with too many of her body parts.  All it takes is one to go the wrong way...

I have never thought of her conditions as being "terminal."  Except when I was pregnant with her.  I knew the odds were stacked against her then, for sure.  But, it really seemed like after that, she could beat it.  We've never done anything crazy to keep her alive.  Just a whole bunch of surgeries.  None of them insane.  Sure, her leg bone is in her face.  But, even that's just another surgery day at Boston Children's Hospital.

But this?  When we get to the point where we really need to make a decision about this heart and lung transplant.... that seems insane.

And all my hopes up until now were fixable things.  Like, maybe another, different, jaw surgery will be the ticket to a better airway.  After this heart surgery, her heart will be better.  After spinal fusion surgery, her back will be better.  But, now, I have to hope that her left lung will just be able to handle the workload?  Even though right now, it can't.  At 7, and 46 pounds, it needs help.  But, maybe that will change.  Maybe there will be more medical advancements made in time to help her.

That's ridiculous.  How am I supposed to work with that?

So, that's where I am right now.  I need to find hope again.  And I need to figure out what I'm supposed to do when I hear someone say, "maybe she'll be a _____ when she grows up."  How do you live when you don't know how long you'll have your child?  Will she drive a car?  Get a job? Go to college?  I don't think about any of those things when it comes to the boys.  I just assume, that aside from some tragedy, that they will get to experience all of those things.

And for now, we have no focus.  We're not working towards getting the trach out.  We're not working on eating by mouth.  We're not working on ... anything, really.  For seven years, we've been working on things.  And now, we've stopped.  And I feel lost.  I don't know what to do.

When I was young, I worked at a small, family owned restaurant.  I was a waitress.  And if you ever did that, you might know what I'm talking about.  When the restaurant is really busy, you're on.  You have energy, you can remember stuff, you're working hard and feeling good about it.  But, after the rush, when the restaurant is empty and you only have a few tables, you suck.  You forget to check on them.  You forget their drinks.  You have enough down time that you can sit down for a second and then you realize how much your feet hurt.  Getting up again is that much harder.

That's kind of how I feel.  We've been so busy for so long.  And it slowed and I sat down.  And my feet hurt.  And I'm really freaking tired.  And now rescheduling that appointment with nutrition that we missed a few weeks ago seems an impossible task, that has been on my to-do list for weeks.  Going anywhere takes an exorbitant amount of energy.  And that layer of grief that is always just below the surface, is harder to rise above.

I know I have to figure this out.  Living with this sadness, I mean.  Maybe once school starts, it will be better.  We'll see.

Anyway, back to the pacemaker appointment... Harlie and Brandy returned from their walk and Harlie was definitely breathing heavy.  But, her sats were 84 and her heart rate was 120!  To put it in perspective, when we went to her cardiology appointment in February/March, we were in the waiting room and her sats were 90, on one liter of oxygen.  While in the waiting room at the end of June, her sats were 86, on two liters of oxygen.  After walking, her sats were 84 on two liters (but this was after physically exerting herself).  So, that's huge!

Now, a month later, on July 31st, she rode her bike around the block for the first time EVER.

What a wonderful change!  Granted, with training wheels and she went really slow.  We took the jogger stroller just in case she couldn't make it all the way around.  And we put the oxygen tank and suction machine in that and walked beside her the whole way.  I had to push on her back a little to give her a little help when the road was flat or inclined.  When she went down hill she would go two inches, and then apply the brakes.  It took us about 40 minutes to make it around.  But, she did it!  And she never once asked to get in the stroller, so that's awesome!

Happy Birthday Brandy!

I'm thinking that pacemaker adjustment was life changing.  She's had more energy in the past several weeks than she has had in years.  She actually sits and plays with toys instead of watching TV all the time.  I think she had no energy before - so it was easiest to just sit on the couch and watch TV.  Now she plays!  A few weeks ago, the bike got out.  I don't know how.  We got it for her years ago, and it had never seen the street.  But, she rode it around the house.

What?  Your kid doesn't ride her bike in the house while wearing a cat costume?  In July?

Okay, this has been long enough.  I have so much more I could share, but I am running 14 miles in the morning.  So, I have to get to bed.

Thanks for reading!  And thank you, as always, for your support.  Without my friends and family, I would be a mess!

Much love,
Christy xo