I'm pretty sure I've lost all of my readers in the last seven months. Some of you have found ways to get in contact with me to make sure everything was okay. Thanks for checking in.
I didn't stop blogging. I just got overwhelmed with all the information I had coming in and didn't really know how to put it into words.
So, let skim over the things that don't really matter. There were some hospitalizations. We've actually only spent a little over a month in the hospital since July which is a huge change for us. I think all of our time spent recently was due to infections. One of them was really scary...like internal bleeding scary. The others just required hospitalization because the only IV antibiotics they could use to treat the particular strains of bacteria infecting Aria were nephrotoxic so they needed to closely monitor her kidneys.
Speaking of kidneys, we kind of really need to do something about Aria's bladder and kidney issues. We've been bucking a big open surgery to fix this problem for a few years. The scales of weighing quality of life against medical intervention seem to have tipped in the favor of the surgery. So, that's likely to happen soon. I just need to pull the trigger.
Blood counts are not great but definitely could be worse. Given Aria's current counts, Hem-Onc recently asked me what our plan would be when Aria gets to a point where she's transfusion-dependent. We aren't far off from that and, if you had asked me what our course of action would be when it all seemed hypothetical, I would have had a definitive answer for you. I just said we'd talk about what not intervening would look like when we got there. He let me know that the decision he thinks we will make is a decision he thinks is the right one for Aria.
There was another stroke. We didn't even notice it. It was probably a day where she was just screaming and screaming and seizing and seizing. An imaging study looking for something else picked it up after the fact. My application for "Medically Fragile Parent of The Year" is in the mail.
The nerve in Aria's left eye degenerated. So transplant on that eye, the eye that used to function the best based on ERG and VEP results, is off the table.
Daisy is kicking ass and taking names with all of her therapies.
So, lets talk about the things that I didn't say before July when I stopped writing. This will be fun.
Aria's cornea transplant is covered by scar tissue. She has about a 2mm hole left to see out of with one eye. That hole is covered by some blood vessels and the treatment, an off label use of a chemo drug injected directly into the eyeball, can't be used because of her FA. She still has a tiny bit of vision it that eye which is infinitely more than the amount of vision she had before the transplant. I've been just kind of not talking about that for a year. If she ever loses that entire transplant I'm going to be super pissed about the twelve pounds I gained during transplant follow-up that I can't seem to lose. Priorities.
Aria stopped hearing with her BAHA. I mean, we were never sure if she heard with it or if she was just feeling the vibration since her hearing impairment was right at the limits of the BAHA's capabilities. When she first got her BAHA she was responding to sounds in the normal range in the sound booth but she could have been responding to the sensation. We were happy with awareness of sound even if it was through vibrotactual input because we were trying to avoid putting her through a Cochlear implant surgery (that's the route ENT wanted us to go from the beginning). Also, measuring behavioral responses in a sound booth with Aria is kind of a joke. I made her wear it for like six months after she stopped responding to all sound just so I didn't have to talk about it with people while we explored our options. She hasn't responded to sound in well over a year and I'm not so sure that she ever got anything more than vibrotactual stimulation from that hearing aid. It was fun to bedazzle the headbands while it lasted though.
We eventually went for a cochlear evaluation, another ABR, and MRI and found that her hearing had declined way past the limits of the BAHA. They got no brain stem response on the left and responses on the right in the profound range.
Of course, Aria's health is not the same as it was when a cochlear was initially suggested as the best option so I found myself fighting to improve her quality of life once again. All of the surgeon's concerns were valid but the risks he was considering did not outweigh the possible quality of life benefits to Aria given the fact that her cornea transplant isn't doing great and transplanting the left eye is no longer an option.
He initially proposed implanting on the left side, away from her VP shunt that tends to have a lot of problems, hoping that it would work even though there had been no brain stem response from the ABR. We simply were not going to do that. We would have done bilateral but we were not going to put her through a surgery that looked like it probably wouldn't work. It took coordinating conversations between multiple specialists across multiple hospital systems but, in the end, her physicians agreed that the possible benefits for Aria outweighed the risks. She was cleared for a Cochlear implant.
So, in the end of January, Aria had a Cochlear implant. Aria normally spends most of the winter inpatient because fighting off seasonal viruses is just not something she excels at. I was so committed to getting this implant done that Aria didn't leave the house for over a month unless she was going to her Intervener's pre-sanitized house. All therapies were cancelled and she did not attend school. I did not want to risk her getting a virus and getting the surgery, a procedure that schedules many months in the future, cancelled.
Confession time: The afternoon before her procedure we busted out of the house to go to Disney on Ice and brought Aria with us. I figured, if she caught a virus that day, she wouldn't be showing symptoms by the time of the procedure. This is where I need you to stop judging me because she didn't even get sick from going to hang out in that cesspool of kids. Besides, look at these pictures:
|Mommy and her girls|
|Daddy and his sweet Aria|
|This is so much better than screaming and crying|
|Nice relaxing snuggles before surgery|
Aria has been working hard on healing since the procedure. Often the device is activated about two weeks after the procedure. We are waiting about three times as long.
|Aria stayed in the hospital for one night after the procedure.|
|You should see the other kid.|
|Snuggling Aria-on-versed all the way to the OR in the sassy and flattering little outfit.|
I guess you might be wondering why I haven't discussed the Cochlear implant much.
Before the procedure whenever I would share with people that Aria was getting a Cochlear implant, the overwhelming response was "I love watching those videos of kids hearing with Cochlear implants for the first time!" or "Did you see that video of that kid hearing his Mom's voice for the first time?"
Basically I was afraid that people would start flooding me with these videos or stories of people they know who communicate very well as a result of their cochlear implant.
You guys. That's not going to happen.
Not only is that not going to happen but that has never even been our goal. For anything. Our goal is to give Aria more access to information about the world around her. We know that her brain stem has responded to auditory input but that says nothing for how Aria will process that sound, whether she will find meaning in those sounds, and if those sounds will translate into speach for her. We aren't pulling any of those possibilities off the table but they are not our expectation. Not even close.
Every family has to go through this cochlear implant evaluation checklist. It took me about nine months to complete everything. Once Aria was officially a candidate based on imaging studies and ABR results we had to go through the last step of the pre-cochlear checklist. It was a psychological evaluation for the family. We had to go into a psych ward with our kids so that we could be evaluated to make sure we had appropriate expectations and that we had a thorough understanding of and willingness to followthrough with the shockingly small number of post-implant appointments. The psychiatrist was nice enough but I wanted to throat punch her the entire time. I had a feeling that a throat punch would automatically get my kid disqualified so I didn't dole any out. The idea of sitting in front of someone who has never even met your child before while they try to explain your own child's limitations to you and then evaluate how well you take it, as if any of these limitations were news to you, is laughable. It was a waste of time and money but a necessary evil, I guess.
Frankly I was afraid that allowing the peanut gallery to weigh in on Aria's Cochlear implant with their unrealistically high expectations for results or - worse yet - warnings that activating a Cochlear for the first time often is not a good experience for children would make me want to throat punch you too. And we are all friends here. I don't want to throat punch my friends.
My expectation is that Aria will become aware of sound once again like she was when she initially got her BAHA. My hope is that she will startle less when people approach her. My dream is that she will one day hear me tell her that I love her and that those doting sounds will provide her a better understanding that she is loved beyond words.
Our expectations are simple and I feared that allowing others into the process would make it more difficult than it already has been.
Aria's device gets activated in two days. Here's hoping our expectations are met.