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.
