Excuse the lateness of this post it has been hard to write and the kids started school this week so it's been a busy week.
Wednesday our nurse and I headed to Children's for voice clinic after taking the older kids for their first day of school. I'd heard about voice clinic by chance and was able to get an appointment after much red tape since Ainsley isn't quite the required 3 years old yet. I've been looking forward to it for a few months, hoping this might provide some answers about whether Ainsley will be able to speak one day. Though at the end of the appointment I found out that this "voice expert" was not particularly experienced with trachs, still we got some useful suggestions and after much talk with her and the ENT, and thinking things over since, I think I have a clearer picture of Ainsley's situation and what we can do to help her.
Of course just like at the last appointment she was virtually silent. She seems to clam up when she gets in front of the professionals. I tried to get her to "talk" for the therapist who was there to evaluate her voice and felt like a complete fool. On the way home in the car she couldn't keep quiet. Why oh why didn't I bring video to show?! That was just the beginning of the frustration.
Ainsley's vocal cords and arytenoids are scarred which makes them somewhat rigid and unable to move easily. It also causes her voice to sound different (kind of hoarse and strained). It is harder for her to move them adequately for breathing which, in effect, reduces the size of her airway at the point of the vocal folds. Although she could probably manage without the trach most of the time she runs into trouble when she cries because the cords slam shut and don't allow air to pass. She could also have trouble while sleeping (this often happens) because during sleep the brain isn't as likely to help compensate for breathing difficulties, such as moderating breathing patterns, like it would while she's awake. This is why Ainsley still has the trach even though the airway edema has mostly resolved since the Nissen fundoplication last October.
Ainsley's ENT had told us in June that she may never speak, probably because he heard no vocalization at that appointment and knew she had a lot of scarring when he looked during the laryngoscopy he'd done in March. At this appointment he didn't remember saying that, which was frustrating since we obviously took the implications of that quite seriously, even though to our ears it seemed she was physically able to vocalize despite the obvious fact that it doesn't come easily to her. Again he said her voice may sound different (which it clearly already does) and she may not have enough air to speak an entire sentence without pausing. He also has said on both occasions that kids will find ways of compensating and make sounds using other parts of the airway. Although I have to wonder if this takes a certain amount of cognition that may be beyond Ainsley. Regardless, even with this severe scarring we can now rule out the anatomy as the sole cause of her lack of speech. Which is good.
Ainsley has been working with a great speech pathologist since she was about 18months old. Despite her efforts Ainsley's progress has been slow in most areas related to communication. She has no words and only signs: Hi/bye, more, all done, book, and has started to use help in the past week or so. Sign language is difficult for her due to her cerebellum malformation and it's effects. The swelling of her airway was so bad that she was unable to wear a speaking valve until last November so she was essentially silent for the first 2 years of her life. Even since then it hasn't been easy for her to make sound. And it is difficult to keep a speaking valve on her trach since she likes to pull it off and put it in her mouth, even chew on it. So the amount of hours she has actually had a "voice" is minimal due to the trach. Since we were never sure if she would have a voice to use we've worked pretty hard in therapy on learning PECS, assuming she would need some form of communication with the world at large. It is likely that she will still need an AAC but after this appointment we now know we should also be working harder on voice and speech.
The main missing component is Ainsley's desire to communicate. She has limited needs and desires and a pretty easy going temperament so she has been able to get her basic needs fulfilled with grunts and gestures (pointing and a little signing). As her cognition increases that may change. We already use mimicking to try to encourage her to vocalize but now we are going to specifically work on trying to get her to mimic vowel sounds. My hope is that if we can spark an interest in vocalizing by making it fun and showing her a taste of success it will motivate her. I'm thinking perhaps I might be able to enlist the kids' help by making up some fun games they can play with her every day. There are so many things to do in our busy life, I need some help.
The doctor was surprised again by how well Ainsley did wearing the cap in his office although there was a point she was retracting at the stoma, which I pointed out to him. I have no idea what is going on in his head. I realize Ainsley is a unique case and it's complex but he's gone back and forth about some important points. I think there must be some part of this that is not adding up in his head because of the way he observes her in clinic as if he can't quite believe she is capped. Perhaps he will figure out something during the laryngoscopy she will have in two weeks at the same time as her tonsillectomy/adenoidectomy. I still hope that there is more obstruction from the tonsils and adenoids than anyone has been aware.
The therapist indicated her professional opinion was that there is no need to rush to do anything (surgically) since she is physically capable of vocalizing. I had to disagree and point out that clearly having to rely on a speaking valve to make sound and only being able to vocalize part of the day must clearly have an impact of some kind. In addition to the strain of not having normal airflow through the cords. But this is where a speech expert with trach experience would be of benefit. The deck is stacked against Ainsley and I think having non-ideally functioning vocal cords and a trach just makes it harder for her to learn to use her voice. Although I think that in many cases kids with trachs can manage quite well with a speaking valve it's not ideal for Ainsley.
She indicated that traditionally they think of speech following eating in development of the muscles of the mouth and tongue. It doesn't help that Ainsley does not eat orally but there is not much that can be done on that front. I've tried as much as I can. Clearly there are kids that speak that don't eat so I don't think that's the answer. She questioned whether Ainsley has an oral aversion that is causing her to not eat. Despite professional opinions to the contrary, I still believe she does not because she puts everything in her mouth including some foods. She seems to be able to tell when she might gag or aspirate on something she's trying to swallow. I think it's self-protection. I'm still hoping that the tonsil removal is the answer to getting her eating. But I think it's not a bad idea to incorporate some more oral exercises (vibrating toys, chewy tubes, textured objects, whistles etc.) into our routines.
The therapist brought up the fact that kids with low tone can have nasal sounding speech after an adenoidectomy. A fact the doctor had already informed us of last month during the consultation about removing them in the first place at which time we agreed he would only partially remove them. Best to be safe. He proceeded to explain it again as if it were the first time and even went so far as to amend the surgery consent form. Scary. I assumed since he knew it was to be a partial the degree of removal didn't need to be specified. So let that be a word of caution to all of you that you can't be too specific in those surgery consent forms. I hope that he would have referred back to his notes and remembered he was only performing a partial since the consequences of such a mistake could affect Ainsley's voice for the rest of her life. It shook me up a bit and in combination with some of the other serious things he's forgotten in the past I would be lying if I didn't admit to being a bit nervous. However he is a highly respected surgeon. Dr. Cotton's office, who had seen Ainsley in the past and is considered by many to be the top in the field of otolaryngology, has told us we are in very good hands. I am hoping he can work some magic when he's in the operating room even if paperwork isn't his strong suit. Please pray that he gives her entire situation serious consideration beforehand and that on Sept. 28th he makes guided decisions about what is best to allow Ainsley to have a safe airway and a voice.
Here's a little video of Ainsley's "voice" right now: