Happy New Year!

Zadie's 2012 consisted of too many ER visits, five bouts of pneumonia, one very nasty case of RSV, two surgeries and a lot of learning and growing and fun!  We got great news when I checked the mail on Christmas Day (mail that had been delivered the day before) and got the letter from the insurance company that our second level appeal had been approved and Zadie was now back to 16 hours a day, 7 days a week of private duty nursing.  We are in the process of trying to get the shifts staffed.  I look forward to sleeping in a real bed, since my air mattress seems to be losing air quicker and quicker every night.  It might have to do with the 90 pound dog that likes to join me sometimes or the cats that like to jump around on it.  I also look forward to Zadie being able to be home during the day, rather than coming to daycare with me and Jonathan.  It's nice for her to get some socialization, and it's really nice to see her all day, but her health is more important right now, and I'm afraid that she will have a very rough winter if she continues to come with us.  

Zadie learned all sorts of stuff in 2012.  She learned to walk and sign and she even gained back her voice!  It's not 100%, but she can get very LOUD when she wants to express herself.  She can also say mama and baba meaningfully.  She got her palate repaired back in May, and can drink like a champion.  

By 2014, I would love it if Zadie could lose her trach and her Gtube.  I won't get my hopes up or put too much pressure on anyone, but it would be great.  For now, she is happy and healthy and that's all we can ask for.  

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?!?

Meeting with ENT at CHOP...

BJ and I took Zadie down to CHOP today to see Dr. Javia, the ENT.  We had met with him in December and he prescribed the Passy Muir valve (PMV - speaking valve) at that time.  He wanted us to come back to check the pressures to make sure that it was safe for Zadie to use it.  She ended up being sick a lot from January to April, and then had her palate repaired in May, so we didn't get back to him.  Besides, we had pretty much decided to stick with St. Joe's for all of her care, so we didn't really plan on going back to CHOP.

Well, now that I'm not happy with the ENT at St. Joe's, and the craniofacial team there kind of dropped the ball on guiding us through all of this, we're kind of up in the air again.  I feel terrible that Zadie is still not able to make sounds.  My hope for the ENT visit today was make sure that the PMV was safe to use and then we could get working on it.  Unfortunately, since no one has ever looked at Zadie's airway, the doctor wants us to hold off on the PMV for now.  We have scheduled a bronchoscopy, which is a scope of her airway and is done while under general anesthesia.  That will be happening on September 4, which is just a few weeks away.  I'm anxious and excited to find out what's going on in her airway.

The doctors at CHOP are very fixated on doing a jaw distraction on Zadie.  We are hoping it won't be a necessary surgery, so we aren't entertaining the notion right now.  The fact that they are so gung ho about it is a big reason we shied away from  CHOP in the first place.  If we can avoid two additional surgeries (one to place the distractors and another to remove them), plus all the rough stuff to get through the procedure, plus all the risks involved, we would love to do so.  It's not clear to us at this point that it's a necessary procedure.  Maybe after the bronchoscopy, things will be clearer.  I am pretty bummed that today's visit didn't go as I expected.

We are considering getting yet another opinion on a course of action.  We are pretty much resigned to the fact that Zadie will have the trach at least through the winter, but we'd love to be on track to get it removed by the spring/summer of 2013.

On a positive note, Zadie had a hearing test, and did well, so I think the tubes are doing their job.

And on another note, I already know how lucky we are, but being at the Children's Hospital today, and seeing all the sick kids, I was reminded even more about how truly blessed we are.  Zadie's good and happy and I know this all is just a bump in the road.  I'm just looking forward to the day we can look BACK at this particular bump.

One year at home...

Today is the day, a year ago, that Zadie finally got to come home from the hospital! Leaving her there when I was discharged was one of the hardest things I'd ever done! I always heard about babies having to stay in the NICU, and I always said I couldn't imagine how hard that would be. Unfortunately, I found out. What made it a million times worse was that almost every day when I went to the hospital to see Zadie, I'd see a beaming mom sitting in a wheelchair, holding her tiny bundle of joy, waiting for dad or whoever to bring the car around.  It really was like a knife to the heart to see that, every time.  It never got easier.  I often wished that there was some secret NICU parent entrance so we didn't have to pass by that scene every day.  I also realized how lucky I was that I got to experience that once.  I was that beaming mom holding my little Jonathan just three years earlier.  It was a long ten weeks, and I couldn't believe the day had finally come where they would release Zadie!

Zadie with one of her favorite NICU nurses, Patty

Zadie's neonatologist, Dr. Ruben, sending her off

Proud big brother with his baby sister home for the first time!

It was torrentially raining that day, and when we got home, the director of nursing of the home care agency and four nurses were waiting to be oriented on Zadie's case.  From that day on, we've had nurses in the house 16 hours a day almost every day.

So much has changed in the last year!  Zadie has grown, of course, and is happy and healthy and on the go.  She's been back in the hospital a few times.  She no longer has a cleft palate.  She's eating and drinking by mouth, although still uses her tube overnight (mostly because we haven't gotten the orders changed).  We are old pros at changing trach ties, changing the trach itself, getting it back in if it gets pulled out, changing her Mic-key button (feeding tube), putting that back in if it gets pulled out.

Maybe, just maybe, in a year from now, Zadie will have put a lot of this behind her!