Sunday, September 30, 2012

Zadie's getting noisier!

We took Zadie to CHOP in the beginning of September for a bronchoscopy.  At the time, they downsized her trach because the ENT saw that she has a very small airway and her previous size trach was pretty much filling up her trachea, which is why she never made any noise.  The hope is that with a smaller trach, she can tolerate a Passy Muir valve (PMV), which will allow her to start vocalizing.  The day after her bronchoscopy and stoma revision (where they removed some scar tissue and fixed her trachea, which had partially collapsed), we tried the PMV while still in the hospital.  She must have still been swollen from the surgery because she did not tolerate it well at all.  We made an appointment to come back to the office in October (a week from tomorrow!) in hopes that the swelling goes down enough for her to tolerate the PMV.  If she still doesn't tolerate it, we will downsize again.

Anyway, gradually over the past few weeks, Zadie has started to make noise!  It's not all the time, but she does get squeaks out!  Sometimes it's when she's crying, but sometimes it's when she's happy and she's clearly intentionally making noise.  This is a great sign, because that means she's pushing air up past her trach and out her mouth and nose.  I think we have a good chance of her tolerating the PMV next week.  And if she doesn't, then I think the smaller size trach will really do the trick.  I hope so!  It's really, really great to hear her making sounds.

In case you missed my blog post from earlier this year about why she's so quiet, here you go: Why My Baby is So Quiet.

Sunday, September 9, 2012

First bronchoscopy

Zadie had her first bronchoscopy on Tuesday.   It's done under general anesthesia and it gives the ENT a good look at her airway and vocal cords.  This was Zadie's fifth time being under general anesthesia.  It's never easy to send your little girl off to be put under!  We have to put a lot of faith into the doctors.

Jonathan had come down to CHOP with us, and BJ was entertaining him while I stayed with Zadie during the pre-op period.  The ENT told me that it would take anywhere from 15 minutes to an hour.  15 minutes if he looked in her airway and everything looked good.  It would take longer if there was anything that needed to be done, such as cutting out granuloma (scar tissue) or stitching up a collapsed trachea.  I was nervously waiting for it to be done and watching and hearing other doctors come out to talk to parents in the waiting room about how great their kids did.  The lady running the waiting room came to get me and said, "the doctor is ready to talk to you" and took me to a private room!  She didn't say anything and just left me there.  I really started to freak out - why didn't she say Zadie did great (I heard her tell other parents that) and why didn't Dr. Javia come out to talk to me in the waiting room?  After a good five minutes of increasing anxiety, Dr. Javia finally came in, all smiles, saying she did great.  He said, "I didn't scare you, did I, putting you in the room?"  Uh, yeah you did!

Anyway, Zadie did do great.  He did have to cut out a granuloma, which had been causing her airway to be 50% obstructed!  Her trachea was also partially collapsed, so he put two stitches in to take care of that.  Because of that surgery that had to be performed, they admitted her for the night.  The best part is that because her airway is very small, and the size trach she had in filled up her airway, the doctor downsized her.  This is great because it will allow more airflow past the trach and up through the vocal cords.  This is a better chance for Zadie to make noise, start talking, etc!  I am so happy that we decided to get another opinion and not just listen to whatever one doctor told us.  If we had listened to the ENT at St. Joseph's, she would not have had a bronchoscopy for another 2-3 months, and who knows how obstructed her airway would have become.

Since her trach is now downsized, she has a better chance of tolerating the Passy Muir valve.  This is a special valve that goes on the trach.  Air can flow into it, but not out of it.  Therefore, it forces the person wearing it to breathe out her mouth and nose.  Zadie's never been able to tolerate it, but now we know why.  She had a big obstruction in her airway and her airway is very small and was completely filled up with the trach.  If there's no airflow past the trach, then the valve won't work.

So, on Wednesday, we tried the Passy Muir again, with a nurse practitioner from the ENT office observing and testing the pressures with a manometer.  There are certain parameters that they look for as far as the pressure, and if the pressures are too high, then the valve isn't being tolerated and can not be used.  Unfortunately, Zadie didn't tolerate the valve well at all.  The NP said not to get too upset about it.  Zadie might still be swollen from the surgery and we will try again in another month in the ENT's office.  If she still doesn't tolerate the Passy Muir then, we will downsize the trach again.

This is all very exciting, because these are all steps towards decannulation (getting rid of the darned trach)!  It's not in our very near future, but it is there.  She'll definitely have the trach at least through the winter, but maybe next summer?!?