We had an appointment today with the speech pathologist. (Yes, that one.) It went far better than I expected. In fact, we now have solid evidence that not only is Namine speaking better with the palatal prosthetic in, her speech is improved when she isn’t wearing it.Most of the resentment and ill will I bore toward the speech pathologist was due to his not looking at the bigger picture. As you may (or may not) recall, he vouched for having Namine’s frenulum (the webbing under the tongue which connects it to the floor of the mouth) clipped.

Let’s back up a bit.
(If you remember everything that’s happened or you don’t want to read back-story, you can skip ahead to just read about today.)

Three major things came about as a result of Namine being born with Pierre Robin Sequence. First, she had a regressed jaw. Precisely why, we do not know. And nor will we ever; we might as well ask why she was born female. But this started a chain reaction: the sequence named after one Pierre Robin. The regressed jaw affected her tongue’s placement and development, causing ankyloglossia (being tongue-tied) and also causing it to be in the way of the formation of her hard and soft palates. This, in turn, resulted in a partially cleft palate – invisible when looking at her face, it formed a U shape space through her hard and soft palates.

Namine loves looking at her baby pictures. "It's baby me!"
Namine loves looking at her baby pictures. “It’s baby me!”

Because of all this, Namine could not breathe or eat on her own, resulting in her tracheostomy and g-tube. The surgeries that allowed her to be rid of the trache and g-tube were the mandibular distraction and cleft palate repair. But even though the modifications to her mandible and palate allowed her to breathe and eat on her own, her jaw still sits further back than it would normally. This is a crucial detail, one overlooked by our esteemed speech pathologist: if the frenulum were to be clipped, it would free her tongue (which, remember, still sits further back in her mouth). It would free it, but there is a good chance that it would collapse into the back of Namine’s airway. Two possible solutions exist, at that point: another tracheostomy (with which she cannot make much noise) or stapling her now-freed tongue to the floor of her mouth (once again restricting it from movement). But all that is in the past. We vetoed the whole frenulum-clipping thing, with the support of Namine’s ENT (ear-nose-throat) doctor. But what the doctor was in favor of was the palatal prosthetic. I should point out that the palatal prosthetic is not a replacement for clipping the frenulum. There are two problems, not one, that vex Namine’s speech. The first, which clipping the frenulum would solve, is the fact that her tongue cannot move in all the way it needs to in order to create proper speech. The second is the air leaking out of her nose when she makes consonants, which is solved – or improved, at least – by the prosthetic. I’ll give you a couple examples. An S sound is made by placing the tip of the tongue against the back of the front teeth. Namine’s tongue cannot reach, so she approximates by raising the middle of her tongue to the roof of her mouth, resulting in something in between S and SH. It’s not a clear sound, at any rate, and wearing the prosthetic cannot help. But when Namine makes a B sound – a consonant made in the front of the mouth – she loses air out of her nose, reducing the quality of the sound. It is here where the prosthetic helps; by partially closing off the pathway to her nose (but not completely, so she can still breathe through it), the quality of sound is increased and she can enunciate more clearly.

Sparkly!
Sparkly!

Namine was fitted for the prosthetic before her third (and hopefully final) heart surgery, the Fontan. But she didn’t actually start wearing it until about a month ago, thanks to post-surgery complications. Since she’s been wearing it for a month, it was time to see the speech pathologist once again.

On to today’s appointment

The plan for today was simple. First, a speaking test; second, a video x-ray. The pathologist had Namine say some designated words while wearing what she called the “talking hat” – a piece of equipment that the pathologist strapped onto her head, which measured the amount of air expelled through her nose while she spoke. Since gaining the ability to truly speak once she was decannulated, Namine has leaked quite a bit of air through her nose when speaking. The results of the test proved to us what we already knew to be true: Namine has substantially less nasal leak with the prosthetic in. (We’ve never been able to hear the nasal quality of her voice about which the pathologist has told us, but we can hear the increased clarity of her consonants.) The video x-ray was more of the same, except different: I held a microphone in front of Namine while they took a video, and she said of bunch of words for them. Then I took her prosthetic out and she repeated those same words for comparison. And something which I have suspected for a while was confirmed by the pathologist: Namine’s speech with the prosthetic out is nearly as good as it was when it was in. The speech pathologist explained to us that there is anecdotal evidence (although no hard data) of patients with palatal prosthetics who experience speech improvement merely through wearing the prosthetic. Namine has not had the prosthetic long enough yet to see any long-term results, so we’ll see the pathologist again after another three to six months for another speech test. All of this is good news. Namine is tolerating her prosthetic extremely well – even up to eight hours – with no complaints of discomfort or pain. Her speech, even while not wearing the prosthetic, is improving every day. But there’s always a catch, isn’t there? The underside of this particular cloud is that every time Namine wears the prosthetic – even as little as an hour – she gets tired. I don’t just mean oh, yawn kind of tired. I mean she gets so exhausted that she will sleep an extra six hours and still be tired. And we don’t know why. What first comes to mind is whether the prosthetic is interfering with Namine’s oxygen and heart rate. But her O2 is the same as it has been since the Fontan – averaging a solid 96 percent. Her heart rate is a little elevated, but only by about 10 beats per minute; she normally runs at about 120 beats per minute, and with the prosthetic in she’s only averaging 130. Her respiratory rate isn’t increased at all, and there are no visible signs of distress or increased effort. To top it all off, there was nothing immediately telling from the video x-ray. The speech pathologist will be passing it on to the ENT doctor and the entire cleft palate team, so we’ll see if they have any ideas. In the meantime, the pathologist has a theory. Namine is louder now than she ever has before – all the time, not just when she’s wearing the prosthetic. This is evidence of her speech improving, of course. But what we’re not seeing is the extra effort for that improvement. She’s still getting the same amount of oxygen, so she must be expending extra energy in order to be louder – and that greater energy cost is going to be recouped somehow, leading to her needing more sleep.

Therapy + prosthetic = one tired Namine.
Therapy + prosthetic = one tired Namine.

As far as theories go, this makes sense to me. So instead of having Namine wear the prosthetic every day, she will wear it every other day, so that she can have a day to rest and recover that lost energy. Over the coming months, we expect to see her body acclimate to its new redistribution of energy, and she should require less sleep – or rather, require the same amount of sleep as she did prior to getting the palatal prosthetic. This will not be a quick change, I expect, but well worth it in the end.