Hearing test

I took Zadie to get a hearing test on Friday.  Her ENT at CHOP wanted it done, and rather than trekking all the way to Philadelphia, we were able to visit their specialty center in Princeton.  

The audiologist checked the placement of Zadie's ear tubes first.  The left one was in place and unblocked.  She could see the right one in her ear, but it's either blocked or not in place.  Therefore, she has some hearing loss in that ear (which is hopefully temporary).  

I talked to the ENT's office, and we are going to try to unblock the tube by dropping hydrogen peroxide into her ear 2-3 times a day for up to a week (or if she starts showing signs of pain, we will stop sooner).  Once we are done with that, we will be dropping ofloxacin drops in her ear for 5 days.  Hopefully this will unblock the tube.  We are seeing the ENT in June anyway, so by then, he'll be able to look in her ear and see what needs to be done.  

In other news, Zadie is doing great wearing her Passy Muir valve.  She wears it all day while she's awake (we take it off for naptime) and she's even building up the time that she wears a cap (which completely blocks the trach and forces her to breathe only through her mouth and nose.)  Since she's been wearing the Passy Muir so much, she's been vocalizing a lot, and is really building up her vocabulary!  I'm hoping that by the time we see the ENT in June, she is wearing the cap all day and we can schedule a sleep study to see if we can get rid of the trach!

Airway Clinic

Zadie and I drove down to CHOP today to see Dr. Javia, her ENT.  He only sees airway patients once a month, and he's not seeing patients in March, so even though I knew we weren't really prepared to go back and see him, I didn't want to have to wait another two months to see him.

There were a LOT of trach babies in the waiting room!  It was very exciting for me.  Zadie didn't really care.  We've seen one or two in the waiting room at other appointments, but today was like a convention.  I've still never seen a trach baby in the real world!

Since the last time Zadie saw Dr. Javia in November, we were supposed to have been working on getting her to wear the Passy Muir valve all day.  She's been sick on and off since then, so we weren't working on it while she was sick.  And she's just really a little stinker when it comes to wearing it.  I've gotten her to keep it on for 15 or 20 minutes at a time, a few times.  But most of the time, she pulls it off after a few minutes.  It's frustrating because we need to get her tolerating the PMV all day and then we can move on to the cap.  She needs to be tolerating the cap all day and pass a sleep study before she can lose the trach.

So today, Dr. Javia said to come back and see him in six months.  My heart dropped, because since the last appointment, I've had spring in my head as a possible time for decannulation.  But, since we haven't really made any progress with the PMV, we're not really any closer.  I am disappointed in myself for not pushing her harder to wear it.  But all I can do is look forward and be tougher with her.  I keep reminding myself that she WILL get decanned one day.

Knocking on wood....

Zadie's been really healthy!  (knocking on wood).  Her brother has been feverish on and off for the past two days, so I'm afraid of her catching whatever it is he's fighting.  But meanwhile, she's been great.  I've been trying to work on the Passy Muir Valve (speaking valve), but she's such a little stinker that she doesn't want to keep ANYTHING on her trach at all.  When we're out of the house, I'll manage to get her to keep the HME on most of the time, but she still pretty much takes it off constantly.  And when we're home, she pretty much downright refuses to wear one.  So when I try to put the PMV on her, it's the same issue.  She'll keep it on for a minute or two and then pull it off and hand it to me.  I talked to the ENT's office today to get suggestions on how to get her to wear it longer, and they just suggested distractions (like TV, iPhone, etc).  Any trach moms have any other suggestions?

RSV & another ER visit

Zadie got a fever out of nowhere last Wednesday.  She had no other symptoms other than excessive sleepiness.  I gave her Tylenol and it went away, but I took her to the pediatrician that day, since pneumonia always comes on fast and is a constant concern.  He listened to her lungs and said they sounded good.  He said it was probably just a virus, but he put her on antibiotics as a precaution so that whatever it was stayed out of her lungs.

She did ok the next day, but then spiked another, higher fever that night, and again on Friday.  She wasn't really eating anything, so I was making sure that she was getting plenty of fluids.  Over the weekend, she was feeling very yucky.  Her breathing was very fast and she was requiring oxygen to keep her saturation levels up during the day as well as at night.  I considered calling the doctor, but I knew that any on call doctor would have us go to the hospital, and most likely, the hospital would want to admit her, and I really wanted to avoid an admission!  So we rode it out for the weekend.  She barely moved from the couch the whole weekend.  She kept spiking fevers, and if we tried to give her anything but Pedialyte or juice, she'd vomit it up.

Monday morning, I called her pediatrician and brought her in that day.  I felt nauseous, because I was positive that he was going to send us to the ER, at least for a chest xray.  And because she had just been in the ER three weeks earlier with pneumonia, I was afraid she was definitely going to be admitted.  I was right about the ER trip, because the ped suspected RSV.  We went right there, and saw our old buddy, Dr. Robbie, the ER doc we've seen way too many times!  Right away, he said she didn't look so bad, especially compared to other times he's seen her, and her pediatrician is just nervous.  He said unless something crazy came up, he wasn't going to admit her.  Huge relief!

The test results confirmed it was RSV, and the chest xray looked normal.  Since RSV is a virus, there's no medication for it, and it just needs to run it's course.  He said it could last 7-10 days, and it's possible that it started last Wednesday with that first fever.

Today was slightly better.  Zadie still slept a LOT, but she did eat a little bit.  She also was smiling more than we've seen in days.  Her breathing was closer to normal, and she didn't need any oxygen.

RSV is a very nasty bug.  At one of our appointments at CHOP, I met a mom and her trached son in the waiting room.  Her son had a trach because of a bad bout of RSV when he was a baby.  Because it's so serious, there is a shot for it, Synagis, that is only given to high risk babies under 2.  None of Zadie's doctors ever suggested we try to get approved for it, for some reason, but the fact that she has a trach makes her a candidate.  I'm not sure if it's worth it to try to get it at this point, but I will ask.

My main goal for the next few months is to keep her as healthy as possible.  We need to be working on the Passy Muir Valve in order to move on to the cap in order to hopefully lose the trach in the spring!  If she's sick, we can't move forward.  Short term goal is to get her feeling good enough to sit on Santa's lap!

It's been awhile...

I went the whole month of October without posting anything!  Quick update...as of about a month ago, Zadie lost all of her nursing hours.  The insurance company says nursing is not medically necessary, that it's simply custodial care, and the only reason she would be granted private duty nursing is if she would otherwise be in a facility.  Our first appeal was denied.  We have a second level appeal to file, which will be done soon.

In better news, yesterday Zadie went back to the ENT to try out her Passy Muir valve (PMV).  This is a valve that forces her to breathe out her mouth and nose, and therefore through her vocal cords, giving her the ability to make noise and vocalize!  After her bronchoscopy in September, she didn't do well on the PMV trial, but it was suspected that she might just still be swollen from the surgery.  Zadie is a little under the weather, so I was nervous that it would affect the attempt, but I didn't want to reschedule the appointment again, since the next appointment would have been in a month, and it had already been rescheduled once.

So, it started out rough because she was very upset.  With her current size trach, the pressures with the PMV on seemed to be too high.  We then changed her trach to a smaller size and tried the PMV again.  After calming her down, she did great!  The pressures stayed in the range they were supposed to and her oxygen saturation levels stayed where they were supposed to!   This is GREAT news.  Besides all the numbers looking great, it was so nice to hear her making little noises effortlessly.  We've been hearing more and more from her, but this was more "normal" baby noises.

The plan now is once she gets over her current illness, we can start working with the PMV every day.  We'll start with short time frames, and then increase each day to get her used to it.  In two or three months, when hopefully she's tolerating the speaking valve during all waking hours, we will switch to a cap.  A cap is just a little piece of plastic that blocks the trach so she'll be forced to breathe in and out of her mouth and nose.  We are shooting for decannulation (removal of the trach) in early spring!  I'm trying not to get ahead of myself, though.  I don't want to get my hopes up too much.  But all is looking good right now!  Now we just have to get through cold and flu season.

I'll post again soon!

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.

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?!?

Why my baby is so quiet!

This is kind of an FYI for anyone who doesn't know much about trachs or hasn't come into too much contact with anyone with a trach.  I would say that describes most people!  It definitely described me before 9 months ago.

The comment I notice most when strangers are remarking on Zadie (other than how amazingly adorable she is!) is how *good* she is because she hasn't made any noise!  I always debate about explaining to them why she's so quiet, but usually I just nod and smile and say thank you.

Zadie is a good girl and she's generally very happy and content.  She doesn't cry often, but she's a baby and she does cry sometimes.  However, because of the trach, she doesn't make any noise except for the stuffy, congested noise coming out of her trach.  It's a pretty sad sight to see your baby crying her lungs out, tears streaming down her face, with no sound.  I can only imagine how frustrating it is for her.

A tracheostomy is an artificial airway.  The hole goes directly from the neck to the trachea, below the vocal cords, so the vocal cords are completely bypassed.  Some people can move air past the tube and up through the vocal cords.  I've heard that kids start to figure out that if they block the trach with their finger, they can make noise.  There's also a special valve that can be worn to allow the trached person to make noise.  This is a Passy-Muir valve.  Zadie has one, and we tried it once, but she didn't do well with it.  She has to learn (or be able to) blow air out her mouth and nose, and she hasn't done that in over 9 months.  So that will take some getting used to, as well as growing on her part, so that there's more room around the trach tube.

Zadie gets speech therapy weekly, and we're encouraged to talk to her a lot even though she's not babbling back at us.  We are also starting to sign with her, so that she has a means of communication until she gets decannulated (gets the trach removed) or is able to tolerate the Passy-Muir on a regular basis.

Needless to say, we can not wait until we can hear our little lady babbling, talking, screaming, whatever, all of the above!

Exciting day...

Today was exciting! Zadie met her new Occupational Therapist and Speech Pathologists from Early Intervention. They all loved her (of course!) and were really impressed with where she is right now. Zadie showed off all her best moves to the OT, including her almost-crawling and pulling up to stand. She also showed off how dexterous she is.

For the speech pathologist/feeding expert, Zadie did really well with a spoon with some applesauce on it. That was the most exciting part for me. It worries me that she takes 100% of her milk by feeding tube. I don't want her to have long term feeding issues. Seeing how excited she was to get that spoon in her mouth gave me real hope that we're getting somewhere! The therapists also said that they can tell Zadie is trying to vocalize. Hopefully soon we'll be able to try the speaking valve out on her.

Next week, we have an appointment with the surgeon at St. Joseph's to talk about a plan for palate repair!