No surprise. The otolaryngologist who in March wanted to wait 3 more months to see if there was any more improvement before going forward with surgery completely changed his tune. I don't know if he forgot that he said "the ball was in his court" to figure out a plan or what happened. Truly I'm stunned by what was revealed today.
Essentially the reason that we are not hearing much sound from Ainsley while she's wearing her PMV is that her vocal cords are not moving well. She has a lot of scarring. That we knew but had been told they could remove scarring with surgery. Well now he is saying that she has severe scarring (i.e. damage/fixation - medical term ankylosis) to the arytenoids and that is why we aren't hearing much sound and this condition is irreversible. He said he is surprised that her voice is as effected as it is by this scarring and on the other hand surprised that she can tolerate the PMV and cap as well as she does. It must just be borderline that there is just enough room for her to breath but not enough vocal cord movement to talk (with the PMV).
So essentially it seems as though there is no way to give her a voice. The surgical options of a cricoid split or partial cordectomy could leave her airway open and vulnerable to infection especially since she micro-aspirates. We could try that route and potentially get the trach out but it could be at the expense of her lungs or maybe her voice.
So we left the appointment feeling very deflated. Especially because this damage is due to the intubation immediately after her birth and we can't help but feel perhaps it could have been prevented. It sounded as if she may never speak and always need the trach.
Ainsley's speech therapist came with us to this appointment to try to get answers about Ainsley's voice. She brought with her a voice output device that we'll be renting. We tried it out while we waited for her second appointment. She loved it and thought it was a ton of fun. I'd brought her PECS book and she was able to identify every picture card when asked to point to it. Cognitively she's starting to understand a lot more. And just as if to prove him wrong she started vocalizing with some modulation of tone. It's not speech but perhaps her vocal cords are not as fixed as he thinks. Here is some video I took:
And look how happy she is here to have a voice. So I guess our plan is to work on sign language and use an alternative communication device and most likely keep the trach indefinitely. Unless she decides to prove him wrong. We'll see him again in the fall. We'll keep using the PMV since she seems to be able to tolerate it and it keeps her airway used to being exposed to air. There is no need to push the cap anymore since it's unlikely she'll be able to be decannulated.
The eye appointment was confusing. The ophthalmologist was against the idea of a tarsectomy and tarsal switch surgery to correct Ainsely's ptosis until the end when I showed him a picture of her eyes before and after her last cranial reconstruction surgery. Then he said yes a frontalis sling made sense for her before but not now (pointing to the photo). But she's right there and I pointed to Ainsley. Why the pictures changed his mind at the end I don't know but after 20 minutes of discussion he said that is the way to go 30 seconds after seeing the picture. He did assure us that though the tarsal switch would be irreversible it wouldn't cause damage to her vision although she may sleep with her eyes open. He thinks it would be best to wait until she is at least 4 years of age when that area is mostly full grown. But at least he agreed that is the way to go. He admitted he is conservative. He doesn't even believe in lasik eye surgery so that says a lot. So now we can make the follow-up appointment with the other surgeon and see if he wants to do it this year or push it out.
In late July we should find out if Ainsley needs the hip reconstruction surgery and if so when.
Since there is the potential for her to have 2 surgeries this year it is probably good to leave her airway alone anyway so we don't risk damage from intubation. Then perhaps in the fall we may reevaluate if we are desperate enough for a chance to remove the trach that we'll try questionable airway surgeries. We know we can do the trach thing forever if we need to, but we are disappointed. At least we know Ainsley will be okay no matter what. She is a happy girl and will remain so even if she can never speak and requires a trach to breathe.