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Wednesday, October 6, 2010

Voicing Our Concern

First let me apologize for my rather prolonged absence from maintaining this blog.  The end of the summer is always a crazy time for me--gearing up for school is not as effortless as it might seem on first glance (you would think that, after nearly fifteen years of teaching I'd have this down to an art but you would be very, very wrong about this assessment).  Add to this the fact that our personal lives got complicated on multiple fronts simultaneously and just hanging on for the ride (let alone maintaining a blog, or doing laundry, or sorting mail) began to seem a superhuman feat.  Within a span of three weeks we fired the nanny we hired to take over the kids' care this year because she turned out to be disastrously incompetent (a nice person but clearly not up to the job and someone who left us worrying about how safe the kids were; if things were this bad when I was at or close to home 100% of the time, I could only imagine how things might be once I was back at work); Sharon's project deadline at work meant that she was regularly getting home after 9pm and sometimes as late as midnight and doing a lot of work on the weekends or evenings---or whatever was left of them, too; and, last but not least, my mom took a major turn for the worse and ended up contracting sepsis and pneumonia and succumbing to these.  In many ways this was a blessing since the last year was one of limbo rather than life for her.  And fortunately I was able to find some extra childcare and get up to see her for a day while she was still more or less lucid.  But still, knowing how independent she was and how much she never wanted to be in the position in which she found herself (if only DNRs were stapled to bodies this never would have happened) made me feel particularly awful for her.  I am sure she is in a better place (even if at the same time I find myself hard pressed to really visualize this 'better place.'  I clearly fit into the better-than-averagely-well-informed-about-world-religions agnostic category in the recent Pew Research Center investigation into US Religious Knowledge).  Anyway, even though I find it a bit challenging to visualize a better hereafter, I certainly was able to visualize the pretty awful reality of the situation she was in.  My brother bore the brunt of her day-to-day care this past year (the nursing home she was living in is close to where he works and not terribly far from where he lives) and I am grateful to him for the time that he and his wife were able to spend with her.  I am not sure I would have been able to be as graceful as they were in the face of the slow wait for death as they were.  So, in the end, it is a relief that she doesn't have to do this anymore.  But none of that makes the experience of losing your only living parent and realizing that you are the older generation now any simpler.  The only silver lining (of sorts) is that I've gotten back in touch with my cousins on both my dad's and my mom's side  (all are much older than me; both of my parents were by far the youngest in their families and married and had us relatively late in life).  That's another long story, but it's sort of weirdly comforting to know that we're all kind of going through the same processes at once.

Anyway, the last three weeks of August were a haze (at best) as a result of all of this, and the semester got off to a rockier start than usual (can you really be five weeks behind when you have merely taught for four?  This is a question I find myself repeatedly asking).  I'm hoping that, now that Sharon's done (for now) with the crazy project that culminated in an all-weekender (seriously.  Three full nights with no more than two hours of sleep is not a good thing) that matters will improve.

And part of that improvement is returning to role of blogger-in-chief.  So, please forgive my prolonged absence.  I'll try to update even briefly from here on out.

So, without further of the Hallie-related matters that we haven't quite managed to stay on top of is related to her voice.  Those of you following our saga know that Hallie's left vocal cord was paralyzed as a result of the PDA (patent ductus arteriosus) ligation surgery that she had when she was two weeks old.  We knew that this was a big risk on babies this small (the branch of the nerve controlling this vocal cord is perilously close to where the surgeons have to implant the titanium clip to close off this open duct and make sure that the blood flows properly through the pulmonary cavity).  But there really was no choice here---this surgery saved Hallie's life. 

Hallie made little in the way of sound when she came home from the hospital and didn't really talk for her first two years and so it was hard to assess what her vocal quality was like.  Over the past two years, though, she's been talking more and these days she's quite the chatterbox.  The only problem is that we really cannot hear what she says if there is any ambient noise (so if we're in the car and she's talking to us from the back seat she is practically inaudible; likewise, if I have her in the front seat of the stroller and am pushing it, I need to stop, go around the front, and ask her to repeat whatever it is that she is saying so that I can respond appropriately).  Hearing her in a classroom full of kids (noisy or otherwise) is not at all possible.  And things are so bad that, at her most recent IEP, the following options were raised:  1. she will need to use a microphone/amplifier system in the classroom full-time; or 2. have her keep up with her sign since it's a lot more likely that ASL will be her primary form of communication given her vocal quality.  I am not opposed to her keeping up with ASL and, if there is no other alternative, I guess we're okay with the idea of using assistive technology to make Hallie's life easier.  But neither seems particularly like a solution of the first resort. Our question is:  what else can we do for Hallie?

This question is a particularly burning one given that communication is already Hallie's weak point.  As a kid diagnosed with Autism Spectrum Disorder, Hallie lacks the innate desire to engage in conversational give and take.  We've been working on these pragmatic language skills in her various therapies and she's beginning to improve a bit (she's still less good at the give-and-take than she is at chattering on about some subject of interest, but we'll take that as a very nice and hard-earned first step).  But we know she finds it frustrating when no one can hear her (she will scream greetings at her peers and they don't respond because they literally have no idea that she is talking; she shuts down a bit every time this happens).  I think that it's some sort of cruel joke that the kid with vocal cord paralysis also ends up on the autism spectrum, but I'm clearly not in charge of doling out the things that people need to go through in this life or they would be distributed in a very different manner....

So, given this situation, we needed to get in to see ENT at CHOP.  We haven't been there in a while (I am not a religious make-my-appointments-every-six-months kind of mom; our time is limited and following up religiously with our seven specialists at Children's Hospital whilst also maintaining Hallie's rigorous therapy schedule is not quite manageable.  So I prioritize and make appointments as I deem necessary).  Hallie had mostly been seeing ENT for ear tubes and check ups on their status and had not been scoped in a while (the last time was in February of 2009 when she had dental surgery).  Since scoping is not something that any of us (and especially Hallie) find fun, it's not like I've been clamoring to get in to have a doctor shove a tiny camera down her throat.

But the time had come to do something.  When I called CHOP last month, they weren't sure whether Hallie belonged in the airway clinic (for kids on oxygen or who are trached) or the voice clinic (for kids with dysphonia--or disorders of the voice).  My strong sense was that the latter was a better fit:  Hallie doesn't have stridor and breathes well (at least when she hasn't contracted pneumonia at preschool) and, while she has some swallowing issues, these are more sensory than anything.  She does, however, have a crappy voice (think a very raspy and faint Stevie Nicks or Tom Waits).  The problem is that she needed to be able to follow precise directions in terms of producing speech sounds (vowels, words, etc) and, while the kid still has some echolalia (incessant repetition of words and phrases) she isn't always hugely consistent in terms of following directions.  Moreover, and this was a big one, it seemed from my conversation with the nurse who runs the Voice Clinic at CHOP that they would automatically be scoping Hallie to visualize what was going on with her vocal cords.  While I am all for scoping if there is a purpose to it (such as:  if we don't scope her will we miss seeing vocal cord nodules or polyps that are dangerous to her) but not necessarily keen on scoping for the heck of it.  We've finally given up the habit of vomiting around here (I think we can count on fewer than two hands the number of times Hallie has vomited this year) and we don't want to do anything to set off a bout of reverse peristalsis any time soon. So I made the appointment with some trepidation.  Hallie was none too thrilled with the idea of going to see "Dr. K..." but a judicious bribe (it cost us the price of a pair of very large helium-filled Mickey and Minnie Mouse balloons and a couple of packs of princess and Tinkerbell silly bandz) sealed the deal.  So it was off to the ENT with Hallie last Friday morning.

Anyway, I think the appointment with ENT went really well.  Hallie was very cooperative in producing the sounds that she needed to for the speech therapists (they are electronically assessing kids' speech at CHOP, which apparently no one else has done thus far) and couldn't have been better behaved.  We had some real qualms about scoping Hallie given all of her issues with food/swallowing that are in no small part related to her history of long term intubation and OG tube usage (on some level I think she 'remembers' the 9.5 weeks she spent on a breathing tube and the even longer period of time she was OG-tube fed and that this helps explain some of her eating issues).  Happily, the doctor in charge of the Voice Clinic at CHOP concurred and did not insist on us doing this.    So we avoided having Hallie vomit all over the professional staff and us and having to deal with the trauma that all of this would have brought to everyone involved.

But the really good news is that there are things that we can do to improve Hallie's voice now and that we need not wait until puberty (or close to puberty) to do so.  As I suspected, the scientific research that is already out there and published is dated.  While it used to be the case that doctors did not surgically bulk (by injecting materials like collagen or teflon or one's own autologous fat, which in Hallie's case would be a bad idea since she has so little to spare) the paralyzed vocal cord and maximize the voice that way, now they are doing this.  Or, more correctly, they are now doing this at CHOP.  This procedure is done under general anesthesia for kids (for adults and probably adolescents, it's often done under local so that the ENT/Speech pathologists can ask the patient to produce sounds as they bulk the cord.  This gives them optimal voice output.  But clearly the trauma of having a doctor bulk your vocal cord via a robot that is attached to a camera that you have swallowed is a bit much for kids so they do this under general for them.  This was a relief to hear, really).  The upside of this is that the procedure carries only a small risk with it (that of anesthesia), involves no incisions and no bleeding/heeling, and allows the patient to go home that day after recovering from the anesthesia.  So it's only slightly more invasive than ear tubes.  It also means that the voice sounds better immediately (not normal, but better).  The downside is that the fix is quite temporary (may last a year or two) and will have to be repeated quite frequently.

The other option is nerve reinnervation.  This should restore the nerve that controls Hallie's paralyzed vocal cord's function.  The non-functioning nerve is first tested to make sure that there is no chance that it is working (that there's no electric impulse in it) and then ENT severs it.  She then cuts a functioning but non-essential nerve and reattaches it to the vocal cord.  Over time that nerve should take over some of the function of the non-working nerve.  While the vocal cord won't operate completely normally, some function should be restored to it and the vocal output should be near normal and the fix should be permanent.  The downside of this is that it takes month for the new nerve to take over, and so ENTs tend to bulk the vocal cord while they're doing the surgery so that the voice will sound better immediately. And the other, more important downsides are:  1.  This is a real surgery that involves an incision, scar, and hospital stay and more potential complications in terms of healing and infection.  The scar should be small and certainly Hallie has other scars, but this one will be fairly visible.  And obviously it is emotionally very hard to decide to have your kid's throat slit open.  2.  This is a new surgery for kids.  There is a long literature on this surgery for adults (it was pioneered over 20 years ago at UC-Irvine) but there are only ten kids who have ever received this surgery and it's being done at only one place and by one pediatric ENT-- the person we see at CHOP.  The youngest child she ever operated on was 2.5 and she would not do that again and the oldest was 8 and there have been a bunch around Hallie's age. But still there are only 10 kids who have had the surgery.  We know that it is quickly becoming the state of the art choice for adults and has overtaken bulking because it's a permanent fix, but still.... 

We can certainly wait and see.  Hallie's voice will not get any better, though.  And there is the very real potential that she will do it lots of damage (she basically screams to be heard and this can cause polyps and nodules and other nasty stuff, as well as teach her bad habits that will make her worse even worse in the long run).  Speech therapy for increased vocal production will likely not be of much benefit to her---her voice is pretty much what it is.  If she does develop nodules or bad habits, that will make the outcomes of any reinnervation surgery less positive.  Thus, doing nothing does not completely appeal to me (though Sharon and I have yet to fully discuss this).  And I'm not thrilled by the idea of repeated bulking surgeries, either.  But, like I said, it's hard to make a decision to slit your kid's throat.

So, in the short - term the goal is to call the mom of one of the kids who had this done (and who is willing to talk to other parents) and find out what she thinks about the whole procedure in retrospect (caveat:  we know she's pleased).  And I am sure we can ask to speak to other parents (I'm not sure they'd want to speak to us, though).  And we can read up on this procedure and learn more about it (I somehow find more knowledge comforting).   And we can always take the middle road and fence sit a bit (historians LOVE to do this):  we could do one bulking and wait a couple of years until this surgery has been done on more kids (even if it is still just being done by this one doc at CHOP).  From what I've read, there is little chance that it will ever be performed laproscopically (you simply have to see too much and check on too much when dealing with isolating and cutting tiny nerves in the neck) but still, more experience is probably a good thing.  That said, both Sharon and I find ourselves oddly at ease with this particular ENT and for whatever reason really trust her (this is not our first experience with her---she has seen Hallie a few times over the past four years and was the first ENT that ever scoped Hallie after we sprung her from the NICU).

Anyway, that's all for now.  If anyone has any opinions, do weigh in.

And, like I said, there will be more (can there really be fewer?) updates soon and some pictures of the kids, who are both doing great, soon.


Unknown said...

Abby - I'm so sorry about your mom - it's such a hard thing to lose a loved one, even if you know in your heart it's a welcome relief. I'm glad you are finding some options for Hallie's voice. Although I don't envy your decision at all! But at least their is hope, and hope, I have found, goes a long way.

Cora said...

I am very sorry to hear of the loss of your mom. Even though she was sick, it is still very difficult.

I'm also glad that you've gotten some options to help Hallie with her voice. Good luck deciding what to do!

Anne said...

It must be such a hard decision to make about which procedure to choose. I am glad though to hear that there are options to treat Hallie's vocal chords. Could you try the bulking procedure first to see how long it lasts before you have to repeat it?

BusyLizzyMom said...

I am so sorry to hear about your Mom but it does sound like she is in a better place with her dignity and in comfort.
Choosing what to do for Hallie is a big decision, I might agree with Anne and choose the least invasive procedure first. Elizabeth has had many offers for invasive stuff and I try to keep the acceptances at a minimum until she is old enough to give her input /choice.
I too don't agree with the doling out of diagnosis's our kids get, I really feel they should at least get a bit of a break (and it would be nice to not have to worry so darn much).

Trisha said...

I am so sorry to hear about your mom. I hate CVA's. And I am sorry if she had to endure a code if that's not what she aweful.

And once again, how awesome are Hallie's moms?! So glad you have options for her sweet voice!